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Editor’s Choice Articles

Editor’s Choice articles are based on recommendations by the scientific editors of MDPI journals from around the world. Editors select a small number of articles recently published in the journal that they believe will be particularly interesting to readers, or important in the respective research area. The aim is to provide a snapshot of some of the most exciting work published in the various research areas of the journal.

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12 pages, 1795 KiB  
Article
Utilizing Clinical Transformation Criteria for Prognostic Stratification in Follicular Lymphoma Prior to Initial Immunochemotherapy
by Yoshikazu Hori, Hiroki Hosoi, Takayuki Hiroi, Ke Wan, Shogo Murata, Masaya Morimoto, Toshiki Mushino, Akinori Nishikawa and Takashi Sonoki
Hematol. Rep. 2024, 16(4), 612-623; https://doi.org/10.3390/hematolrep16040060 - 4 Oct 2024
Viewed by 1765
Abstract
Background: Although the prognosis of follicular lymphoma (FL) has improved, some patients experience early disease progression, including progression of disease within 24 months (POD24). Histological transformation is a critical event in FL. However, the heterogeneity of FL tumors makes it challenging to diagnose [...] Read more.
Background: Although the prognosis of follicular lymphoma (FL) has improved, some patients experience early disease progression, including progression of disease within 24 months (POD24). Histological transformation is a critical event in FL. However, the heterogeneity of FL tumors makes it challenging to diagnose transformation accurately. We retrospectively applied the clinical transformation criteria used for FL transformation assessments at relapse or disease progression to conduct transformation assessments before the initial immunochemotherapy. Methods: Sixty-six FL patients who first received immunochemotherapy between January 2009 and February 2023 at our institution were selected. Twenty-three were clinical-transformation-positive (CLT+). Results: The progression-free survival (PFS) rate of the CLT+ patients was significantly lower than that of the clinical-transformation-negative (CLT−) patients. In the POD24 assessment subgroup, the CLT+ patients had a higher incidence of POD24 than the CLT− patients. There was no significant difference in PFS between the patients treated with CHOP-like regimens and those treated with bendamustine regimens. In the CHOP-like group, the CLT+ patients exhibited significantly lower PFS than the CLT− patients. In the bendamustine group, the clinical transformation did not affect PFS. Conclusion: Clinical transformation criteria may be useful for the prognostic stratification of FL prior to immunochemotherapy. Additionally, they may serve as predictors of POD24. Full article
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9 pages, 3261 KiB  
Article
Comprehensive Genomic Profiling in Non-Myeloid Hematologic Malignancies Identifies Variants That Can Alter Clinical Practice
by Chenyu Lin, Katherine I. Zhou, Michelle F. Green, Bennett A. Caughey, John H. Strickler, Michael B. Datto and Matthew S. McKinney
Hematol. Rep. 2024, 16(4), 603-611; https://doi.org/10.3390/hematolrep16040059 - 30 Sep 2024
Viewed by 1294
Abstract
Background: Comprehensive genomic profiling (CGP) is frequently adopted to direct the clinical care of myeloid neoplasms and solid tumors, but its utility in the care of lymphoid and histiocytic cancers is less well defined. Methods: In this study, we aimed to evaluate the [...] Read more.
Background: Comprehensive genomic profiling (CGP) is frequently adopted to direct the clinical care of myeloid neoplasms and solid tumors, but its utility in the care of lymphoid and histiocytic cancers is less well defined. Methods: In this study, we aimed to evaluate the frequency at which mutations identified by CGP altered management in non-myeloid hematologic malignancies. We retrospectively examined the CGP results of 105 samples from 101 patients with non-myeloid hematologic malignancies treated at an academic medical center who had CGP testing between 2014 and 2021. Results: CGP revealed one or more pathogenic or likely pathogenic variant in 92 (88%) of samples and 73 (72%) of tested patients had one or more mutations with diagnostic, prognostic, or therapeutic significance. The identification of a resistance variant resulted in the suspension of the active treatment or affected subsequent treatment choice in 9 (69%) out of 13 patients. However, the presence of a therapy sensitizing variant only led to consideration of a biomarker-directed therapy in 6 (10%) out of 61 patients. Conclusions: Overall, CGP of non-myeloid hematologic malignancies identified clinically significant variants in 72% of patients and resulted in a change in management in 22% of patients. Full article
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10 pages, 999 KiB  
Article
The Real-World Outcomes of Relapsed/Refractory Multiple Myeloma Treated with Elotuzumab, Pomalidomide, and Dexamethasone
by Hitomi Nakayama, Yoshinobu Aisa, Chisako Ito, Aki Sakurai and Tomonori Nakazato
Hematol. Rep. 2024, 16(4), 593-602; https://doi.org/10.3390/hematolrep16040058 - 30 Sep 2024
Viewed by 1323
Abstract
Introduction: A combination of elotuzumab, pomalidomide, and dexamethasone (EPd) was approved for the treatment of relapsed/refractory multiple myeloma (RRMM) following the ELOQUENT-3 phase II clinical trial. However, the clinical experience with this therapy is still limited. In this retrospective study, we analyzed [...] Read more.
Introduction: A combination of elotuzumab, pomalidomide, and dexamethasone (EPd) was approved for the treatment of relapsed/refractory multiple myeloma (RRMM) following the ELOQUENT-3 phase II clinical trial. However, the clinical experience with this therapy is still limited. In this retrospective study, we analyzed the efficacy and safety of EPd in a real-world cohort of RRMM patients. Patients and Methods: The medical records of 22 patients who received EPd for RRMM at Yokohama Municipal Citizen’s Hospital (Japan) between January 2020 and July 2021 were reviewed. Results: The median age of our cohort was 73.5 years. The overall response rate was 55%. With a median follow-up of 20.2 months, the median progression-free survival (PFS) was 9.1 months (95% confidence interval [CI], 2.5–23.0 months). The median PFS was shorter in patients with a poor performance status (PS) than in those with favorable PS (2.5 vs. 10.8 months; p < 0.01). Patients with prior daratumumab had significantly shorter PFS than those without prior daratumumab (2.1 vs. 23.0 months; p < 0.01). Additionally, patients with prior pomalidomide had significantly shorter PFS (1.7 vs. 10.3 months; p < 0.01). In the multivariate analysis, poor PS (hazard ratio [HR] = 4.1, 95% CI: 1.1–15.6; p = 0.04) and prior exposure to daratumumab (HR = 3.8, 95% CI: 1.1–13.8; p = 0.04) remained significantly associated with shorter PFS. Conclusions: The results of our study suggest that EPd is an active and well-tolerated regimen in RRMM, even in real-world patients. Furthermore, EPd may be useful, especially in daratumumab-naïve patients. Full article
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11 pages, 619 KiB  
Article
Padua Prediction Score and Hospital-Acquired Proximal and Isolated Distal Deep Vein Thrombosis in Symptomatic Patients
by Michelangelo Sartori, Miriam Fiocca, Mario Soldati, Laura Borgese, Elisabetta Favaretto and Benilde Cosmi
Hematol. Rep. 2024, 16(4), 568-578; https://doi.org/10.3390/hematolrep16040055 - 25 Sep 2024
Viewed by 1455
Abstract
Background: Hospital-acquired deep vein thrombosis (DVT) is an important cause of morbidity and mortality. Objectives: The purpose of this study was to evaluate the prevalence of proximal lower limb DVT and isolated distal DVT (IDDVT) and their relationship to the Padua Prediction Score [...] Read more.
Background: Hospital-acquired deep vein thrombosis (DVT) is an important cause of morbidity and mortality. Objectives: The purpose of this study was to evaluate the prevalence of proximal lower limb DVT and isolated distal DVT (IDDVT) and their relationship to the Padua Prediction Score (PPS) in acutely ill, hospitalized patients. Methods: In a single-center cross-sectional study, all inpatients from medical departments with suspected lower-extremity DVT were evaluated with whole-leg ultrasonography during 183 days from 2016 to 2017. Results: Among the 505 inpatients (age 78.0 ± 13.3, females 59.2%) from medical departments, 204 (40.2%) had PPS ≥ 4, but only 54.4% of them underwent pharmacological thrombo-prophylaxis. Whole-leg ultrasonography detected 47 proximal DVTs (9.3%) and 65 IDDVTs (12.8%). Proximal DVT prevalence was higher in patients with high PPS vs. those with low PPS (12.7% vs. 7.0% p = 0.029, respectively), whereas IDDVT prevalence was similar in patients with high and low PPS (14.7% vs. 11.6% p = 0.311, respectively). The area under the receiver operating curve (AUC) for the PPS was 0.62 ± 0.03 for all DVTs, 0.64 ± 0.04 for proximal DVTs, and 0.58 ± 0.04 for IDDVTs. Conclusions: In hospitalized patients, IDDVT had similar prevalence regardless of PPS risk stratification. Adherence to thrombo-prophylaxis in patients was still far from optimal. Full article
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14 pages, 649 KiB  
Review
Blood Microbiota and Its Products: Mechanisms of Interference with Host Cells and Clinical Outcomes
by Luigi Santacroce, Ioannis Alexandros Charitos, Marica Colella, Raffaele Palmirotta and Emilio Jirillo
Hematol. Rep. 2024, 16(3), 440-453; https://doi.org/10.3390/hematolrep16030043 - 6 Jul 2024
Cited by 1 | Viewed by 2166
Abstract
In healthy conditions, blood was considered a sterile environment until the development of new analytical approaches that allowed for the detection of circulating bacterial ribosomal DNA. Currently, debate exists on the origin of the blood microbiota. According to advanced research using dark field [...] Read more.
In healthy conditions, blood was considered a sterile environment until the development of new analytical approaches that allowed for the detection of circulating bacterial ribosomal DNA. Currently, debate exists on the origin of the blood microbiota. According to advanced research using dark field microscopy, fluorescent in situ hybridization, flow cytometry, and electron microscopy, so-called microbiota have been detected in the blood. Conversely, others have reported no evidence of a common blood microbiota. Then, it was hypothesized that blood microbiota may derive from distant sites, e.g., the gut or external contamination of blood samples. Alteration of the blood microbiota’s equilibrium may lead to dysbiosis and, in certain cases, disease. Cardiovascular, respiratory, hepatic, kidney, neoplastic, and immune diseases have been associated with the presence of Gram-positive and Gram-negative bacteria and/or their products in the blood. For instance, lipopolysaccharides (LPSs) and endotoxins may contribute to tissue damage, fueling chronic inflammation. Blood bacteria can interact with immune cells, especially with monocytes that engulf microorganisms and T lymphocytes via spontaneous binding to their membranes. Moreover, LPSs, extracellular vesicles, and outer membrane vesicles interact with red blood cells and immune cells, reaching distant organs. This review aims to describe the composition of blood microbiota in healthy individuals and those with disease conditions. Furthermore, special emphasis is placed on the interaction of blood microbiota with host cells to better understand disease mechanisms. Full article
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23 pages, 707 KiB  
Review
Treatment of Immune Thrombocytopenia: Contextualization from a Historical Perspective
by Daniel Martínez-Carballeira, Ángel Bernardo, Alberto Caro, Inmaculada Soto and Laura Gutiérrez
Hematol. Rep. 2024, 16(3), 390-412; https://doi.org/10.3390/hematolrep16030039 - 26 Jun 2024
Cited by 1 | Viewed by 2863
Abstract
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and inadequate production in [...] Read more.
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and inadequate production in the bone marrow. In this article, we review the treatment of ITP from a historical perspective, discussing first line and second line treatments, and management of refractory disease. Full article
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15 pages, 526 KiB  
Review
Neutropenia in Childhood—A Narrative Review and Practical Diagnostic Approach
by Georgios Katsaras, Silouani Koutsi, Evdokia Psaroulaki, Dimitra Gouni and Pelagia Tsitsani
Hematol. Rep. 2024, 16(2), 375-389; https://doi.org/10.3390/hematolrep16020038 - 16 Jun 2024
Cited by 3 | Viewed by 3454
Abstract
Neutropenia refers to a decrease in the absolute neutrophil count according to age and race norms and poses a common concern in pediatric practice. Neutrophils serve as host defenders and act crucially in acute inflammation procedures. In this narrative review, we systematically present [...] Read more.
Neutropenia refers to a decrease in the absolute neutrophil count according to age and race norms and poses a common concern in pediatric practice. Neutrophils serve as host defenders and act crucially in acute inflammation procedures. In this narrative review, we systematically present causes of neutropenia in childhood, mainly adopting the pathophysiological classification of Frater, thereby studying (1) neutropenia with reduced bone marrow reserve, (2) secondary neutropenia with reduced bone marrow reserve, and (3) neutropenia with normal bone marrow reserve. Different conditions in each category are thoroughly discussed and practically approached from the clinician’s point of view. Secondary mild to moderate neutropenia is usually benign due to childhood viral infections and is expected to resolve in 2–4 weeks. Bacterial and fungal agents are also associated with transient neutropenia, although fever with severe neutropenia constitutes a medical emergency. Drug-induced and immune neutropenias should be suspected following a careful history and a detailed clinical examination. Cytotoxic chemotherapies treating malignancies are responsible for severe neutropenia and neutropenic shock. Rare genetic neutropenias usually manifest with major infections early in life. Our review of taxonomies clinical findings and associates them to specific neutropenia disorders. We consequently propose a practical diagnostic algorithm for managing neutropenic children. Full article
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13 pages, 887 KiB  
Review
Current Therapeutic Sequencing in Chronic Lymphocytic Leukemia
by Samir Mouhssine, Nawar Maher, Sreekar Kogila, Claudio Cerchione, Giovanni Martinelli and Gianluca Gaidano
Hematol. Rep. 2024, 16(2), 270-282; https://doi.org/10.3390/hematolrep16020027 - 30 Apr 2024
Cited by 2 | Viewed by 2522
Abstract
The treatment landscape of chronic lymphocytic leukemia (CLL), the most frequent leukemia in adults, is constantly changing. CLL patients can be divided into three risk categories, based on their IGHV mutational status and the occurrence of TP53 disruption and/or complex karyotype. For the [...] Read more.
The treatment landscape of chronic lymphocytic leukemia (CLL), the most frequent leukemia in adults, is constantly changing. CLL patients can be divided into three risk categories, based on their IGHV mutational status and the occurrence of TP53 disruption and/or complex karyotype. For the first-line treatment of low- and intermediate-risk CLL, both the BCL2 inhibitor venetoclax plus obinutuzumab and the second generation BTK inhibitors (BTKi), namely acalabrutinib and zanubrutinib, are valuable and effective options. Conversely, venetoclax-based fixed duration therapies have not shown remarkable results in high-risk CLL patients, while continuous treatment with acalabrutinib and zanubrutinib displayed favorable outcomes, similar to those obtained in TP53 wild-type patients. The development of acquired resistance to pathway inhibitors is still a clinical challenge, and the optimal treatment sequencing of relapsed/refractory CLL is not completely established. Covalent BTKi-refractory patients should be treated with venetoclax plus rituximab, whereas venetoclax-refractory CLL may be treated with second generation BTKi in the case of early relapse, while venetoclax plus rituximab might be used if late relapse has occurred. On these grounds, here we provide an overview of the current state-of-the-art therapeutic algorithms for treatment-naïve patients, as well as for relapsed/refractory disease. Full article
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11 pages, 1460 KiB  
Review
A 2024 Update on Menin Inhibitors. A New Class of Target Agents against KMT2A-Rearranged and NPM1-Mutated Acute Myeloid Leukemia
by Anna Candoni and Gabriele Coppola
Hematol. Rep. 2024, 16(2), 244-254; https://doi.org/10.3390/hematolrep16020024 - 18 Apr 2024
Cited by 21 | Viewed by 5609
Abstract
Menin inhibitors are new and promising agents currently in clinical development that target the HOX/MEIS1 transcriptional program which is critical for leukemogenesis in histone-lysine N-methyltransferase 2A-rearranged (KMT2Ar) and in NPM1-mutated (NPM1mut) acute leukemias. The mechanism of action of this new class of agents [...] Read more.
Menin inhibitors are new and promising agents currently in clinical development that target the HOX/MEIS1 transcriptional program which is critical for leukemogenesis in histone-lysine N-methyltransferase 2A-rearranged (KMT2Ar) and in NPM1-mutated (NPM1mut) acute leukemias. The mechanism of action of this new class of agents is based on the disruption of the menin–KMT2A complex (consisting of chromatin remodeling proteins), leading to the differentiation and apoptosis of AML cells expressing KMT2A or with mutated NPM1. To date, this new class of drugs has been tested in phase I and II clinical trials, both alone and in combination with synergistic drugs showing promising results in terms of response rates and safety in heavily pre-treated acute leukemia patients. In this brief review, we summarize the key findings on menin inhibitors, focusing on the mechanism of action and preliminary clinical data on the treatment of acute myeloid leukemia with this promising new class of agents, particularly revumenib and ziftomenib. Full article
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16 pages, 604 KiB  
Review
Pathophysiology, Clinical Manifestations and Diagnosis of Immune Thrombocytopenia: Contextualization from a Historical Perspective
by Daniel Martínez-Carballeira, Ángel Bernardo, Alberto Caro, Inmaculada Soto and Laura Gutiérrez
Hematol. Rep. 2024, 16(2), 204-219; https://doi.org/10.3390/hematolrep16020021 - 3 Apr 2024
Cited by 8 | Viewed by 8120
Abstract
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in the platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and impaired central [...] Read more.
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in the platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and impaired central megakaryopoiesis and platelet production in the bone marrow. Here, we intend to contextualize the current knowledge on the pathophysiology, terminology, epidemiology, clinical manifestations, diagnosis, and prognosis of ITP from a historical perspective and the first references to the never-stopping garnering of knowledge about this entity. We highlight the necessity to better understand ITP in order to be able to provide ITP patients with personalized treatment options, improving disease prognosis and reducing the incidence or frequency of refractoriness. Full article
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13 pages, 1284 KiB  
Review
Drug–Drug Interactions of FXI Inhibitors: Clinical Relevance
by Nicola Ferri, Elisa Colombo and Alberto Corsini
Hematol. Rep. 2024, 16(1), 151-163; https://doi.org/10.3390/hematolrep16010016 - 21 Mar 2024
Cited by 7 | Viewed by 3233
Abstract
Inhibitors of the factor FXI represent a new class of anticoagulant agents that are facing clinical approval for the treatment of acute coronary syndrome (ACS), venous thromboembolism (VTE), and stroke prevention of atrial fibrillation (AF). These new inhibitors include chemical small molecules (asundexian [...] Read more.
Inhibitors of the factor FXI represent a new class of anticoagulant agents that are facing clinical approval for the treatment of acute coronary syndrome (ACS), venous thromboembolism (VTE), and stroke prevention of atrial fibrillation (AF). These new inhibitors include chemical small molecules (asundexian and milvexian), monoclonal antibodies (abelacimab, osocimab, and xisomab), and antisense oligonucleotides (IONIS-FXIRX and fesomersen), and thus, they have very peculiar and different pharmacokinetic and pharmacodynamic properties. Besides their clinical efficacy and safety, based on their pharmacological heterogeneity, the use of these drugs in patients with comorbidities may undergo drug–drug interactions (DDIs) with other concomitant therapies. Although only little clinical evidence is available, it is possible to predict clinically relevant DDI by taking into consideration their pharmacokinetic properties, such as the CYP450-dependent metabolism, the interaction with drug transporters, and/or the route of elimination. These characteristics may be useful to differentiate their use with the direct oral anticoagulant (DOAC) anti -FXa (rivaroxaban, apixaban, edoxaban) and thrombin (dabigatran), whose pharmacokinetics are strongly dependent from P-gp inhibitors/inducers. In the present review, we summarize the current clinical evidence on DDIs of new anti FXI with CYP450/P-gp inhibitors and inducers and indicate potential differences with DOAC anti FXa. Full article
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11 pages, 1047 KiB  
Article
Impact of Skeletal Muscle Depletion on Patients with Myelodysplastic Syndrome Treated with Azacitidine
by Eri Takada, Nobuhiko Nakamura, Yuto Kaneda, Kenji Fukuno, Shin Lee, Kei Fujita, Tetsuji Morishita, Yoshikazu Ikoma, Takuro Matsumoto, Hiroshi Nakamura, Junichi Kitagawa, Nobuhiro Kanemura, Senji Kasahara, Takeshi Hara, Hisashi Tsurumi and Masahito Shimizu
Hematol. Rep. 2024, 16(1), 114-124; https://doi.org/10.3390/hematolrep16010012 - 28 Feb 2024
Cited by 1 | Viewed by 1369
Abstract
Background: Azacitidine (AZA) is the standard treatment for patients with high-risk myelodysplastic syndromes (MDS). The impact of skeletal muscle depletion (SMD), which is associated with outcomes of hematological malignancies, on the clinical course of MDS patients treated with AZA was investigated. Methods: This [...] Read more.
Background: Azacitidine (AZA) is the standard treatment for patients with high-risk myelodysplastic syndromes (MDS). The impact of skeletal muscle depletion (SMD), which is associated with outcomes of hematological malignancies, on the clinical course of MDS patients treated with AZA was investigated. Methods: This retrospective, observational study included 50 MDS patients treated with AZA. Muscle mass was evaluated using the skeletal muscle index (SMI), which is the area of muscle mass at the third lumbar vertebra on CT images divided by the square of the height. Results: Of the enrolled patients, 39 were males, and their median age was 69.5 years. Twenty-seven (20 male and 7 female) patients showed SMD. The median survival was 13.4 months in the SMD group and 15.2 months in the non-SMD group, with no significant difference and no significant association between the response rate or severe non-hematological toxicities and the presence of SMD. By contrast, grade 3–4 anemia and thrombocytopenia were significantly more frequent in the SMD group than in the non-SMD group. SMD was associated with severe anemia and thrombocytopenia in MDS patients treated with AZA. Conclusion: Reduced skeletal muscle mass may predict severe hematological toxicity in MDS patients treated with AZA. Full article
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13 pages, 1758 KiB  
Article
Survival Outcomes of Patients with Mantle Cell Lymphoma: A Retrospective, 15-Year, Real-Life Study
by Emanuele Cencini, Natale Calomino, Marta Franceschini, Andreea Dragomir, Sara Fredducci, Beatrice Esposito Vangone, Giulia Lucco Navei, Alberto Fabbri and Monica Bocchia
Hematol. Rep. 2024, 16(1), 50-62; https://doi.org/10.3390/hematolrep16010006 - 18 Jan 2024
Cited by 2 | Viewed by 2825
Abstract
Mantle cell lymphoma (MCL) prognosis has significantly improved in recent years; however, the possible survival benefit of new treatment options should be evaluated outside of clinical trials. We investigated 73 consecutive MCL patients managed from 2006 to 2020. For younger patients <65 years [...] Read more.
Mantle cell lymphoma (MCL) prognosis has significantly improved in recent years; however, the possible survival benefit of new treatment options should be evaluated outside of clinical trials. We investigated 73 consecutive MCL patients managed from 2006 to 2020. For younger patients <65 years old, the median PFS was 72 months and we reported a 2-year, 5-year, and 10-year PFS of 73%, 62%, and 41%; median OS was not reached and we reported a 2-year, 5-year, and 10-year OS of 88%, 82%, and 66%. For patients aged 75 years or older, the median PFS was 36 months and we reported a 2-year, 5-year, and 10-year PFS of 52%, 37%, and 37%; median OS was not reached and we reported a 2-year, 5-year, and 10-year OS of 72%, 55%, and 55%. The median PFS was significantly reduced for patients treated between 2006 and 2010 compared to patients treated between 2011 and 2015 (p = 0.04). Interestingly, there was a trend towards improved OS for patients treated between 2016 and 2020 compared to between 2006 and 2010 and between 2011 and 2015 (5-year OS was 91%, 44%, and 33%). These findings could be due to the introduction of BR as a first-line regimen for elderly patients and to the introduction of ibrutinib as a second-line regimen. Full article
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10 pages, 256 KiB  
Review
Emerging Role of [18F]FLT PET/CT in Lymphoid Malignancies: A Review of Clinical Results
by Anna Giulia Nappi, Giulia Santo, Lorenzo Jonghi-Lavarini, Alberto Miceli, Achille Lazzarato, Flavia La Torre, Francesco Dondi and Joana Gorica
Hematol. Rep. 2024, 16(1), 32-41; https://doi.org/10.3390/hematolrep16010004 - 11 Jan 2024
Cited by 6 | Viewed by 1907
Abstract
Fluorine-18 fluorodeoxyglucose ([18F]FDG) is nowadays the leading positron emission tomography (PET) tracer for routine clinical work-ups in hematological malignancies; however, it is limited by false positive findings. Notably, false positives can occur in inflammatory and infective cases or in necrotic tumors [...] Read more.
Fluorine-18 fluorodeoxyglucose ([18F]FDG) is nowadays the leading positron emission tomography (PET) tracer for routine clinical work-ups in hematological malignancies; however, it is limited by false positive findings. Notably, false positives can occur in inflammatory and infective cases or in necrotic tumors that are infiltrated by macrophages and other inflammatory cells. In this context, 3′-deoxy-3′-[18F]fluorothymidine ([18F]FLT) has been shown to be a promising imaging biomarker of hematological malignant cell proliferation. In this review, a total of 15 papers were reviewed to collect literature data regarding the clinical application of [18F]FLT PET/CT in hematological malignancies. This imaging modality seems to be a suitable tool for noninvasive assessment of tumor grading, also showing a correlation with Ki-67 immunostaining. Moreover, [18F]FLT PET/CT demonstrated high sensitivity in detecting aggressive lymphoma lesions, especially when applying a standardized uptake value (SUV) cutoff of 3. At baseline, the potential of [18F]FLT imaging as a predictive tool is demonstrated by the low tracer uptake in patients with a complete response. However, its use is limited in evaluating bone diseases due to its high physiological uptake in bone marrow. Interim [18F]FLT PET/CT (iFLT) has the potential to identify high-risk patients with greater precision than [18F]FDG PET/CT, optimizing risk-adapted therapy strategies. Moreover, [18F]FLT uptake showed a greater ability to differentiate tumor from inflammation compared to [18F]FDG, allowing the reduction of false-positive findings and making the first one a more selective tracer. Finally, FLT emerges as a superior independent predictor of PFS and OS compared to FDG and ensures a reliable early response assessment with greater accuracy and predictive value. Full article
(This article belongs to the Special Issue Comparison between 18F-FDG and Alternative Tracers for the Assessment of Hematological Ma-Lignancies)
10 pages, 4859 KiB  
Case Report
Plasmacytoma in the Maxillary Jaw: A Diagnostic and Therapeutic Challenge
by Sara Bernardi, Serena Bianchi, Ettore Lupi, Davide Gerardi, Guido Macchiarelli and Giuseppe Varvara
Hematol. Rep. 2024, 16(1), 22-31; https://doi.org/10.3390/hematolrep16010003 - 4 Jan 2024
Cited by 3 | Viewed by 1875
Abstract
Plasmacytoma is a neoplastic disorder originating from plasma cells, with bone and soft tissue being common sites of manifestation. This report presents the clinical and radiological findings of a 65-year-old female patient who presented with an exophytic lesion in the upper right lateral [...] Read more.
Plasmacytoma is a neoplastic disorder originating from plasma cells, with bone and soft tissue being common sites of manifestation. This report presents the clinical and radiological findings of a 65-year-old female patient who presented with an exophytic lesion in the upper right lateral incisor region. The lesion appeared as a unilocular radiotransparent area in imaging tests. Following an excisional biopsy, histological and immunohistochemical evaluations confirmed the presence of mature plasmacellular elements and small infiltrates of B and T lymphocytes. The patient did not exhibit systemic manifestations of multiple myeloma. Surgical intervention, in the form of enucleation of the lesion combined with root canal treatment and apicoectomy, was performed. This case underscores the rare occurrence of plasmacytoma in the jaw region and highlights the importance of surgical management in cases where structural damage or functional impairment is present. Further research on novel treatment approaches is also mentioned, including targeted therapies, immunomodulatory agents, and monoclonal antibodies. The patient is currently under the care of a hematologist for further investigation and the choice of the most appropriate therapy. Full article
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11 pages, 1799 KiB  
Review
Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview
by Gerardo Cazzato, Marialessandra Capuzzolo, Emilio Bellitti, Giovanni De Biasi, Anna Colagrande, Katia Mangialardi, Francesco Gaudio and Giuseppe Ingravallo
Hematol. Rep. 2023, 15(4), 696-706; https://doi.org/10.3390/hematolrep15040070 - 8 Dec 2023
Cited by 5 | Viewed by 2413
Abstract
Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review [...] Read more.
Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review and further studies that deeply investigate this entity not only in a histopathological but also molecular field. In August–September 2023, we searched MEDLINE, PubMed and Scopus for randomized controlled trials (RCTs), narrative and systematic reviews, meta-analyses, observational studies (either longitudinal or retrospective), and case series published in English in the last 25 years using the keywords BPDCN, PDCs, Blastic NK-cell lymphoma, agranular CD4+ NK leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm/tumor. Despite the progress made in recent years in the diagnosis and biological understanding of the disease, until 2018 there was no clear consensus regarding its treatment and the main therapeutic schemes used were based on chemotherapy regimens already used in the treatment of lymphomas, acute lymphoblastic leukemia (ALL) and/or acute myeloid leukemia (AML). In this narrative review, we address the definition and epidemiological features of BPDCN, provide the different theories on the etiopathogenesis with particular attention to the presumed cell of origin, discuss the main clinical manifestations that provide a sign of its presence, summarize the main histopathological and immunophenotypic characteristics with special attention to the most important markers, and finally, we provide some of the most effective information on the therapeutic treatment modalities of BPDCN. Full article
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12 pages, 1268 KiB  
Article
Platelet Microvesicles, Inflammation, and Coagulation Markers: A Pilot Study
by Antonio Gidaro, Alessandro Palmerio Delitala, Roberto Manetti, Sonia Caccia, Mark J. Soloski, Giorgio Lambertenghi Deliliers, Dante Castro, Mattia Donadoni, Arianna Bartoli, Giuseppe Sanna, Luigi Bergamaschini and Roberto Castelli
Hematol. Rep. 2023, 15(4), 684-695; https://doi.org/10.3390/hematolrep15040069 - 4 Dec 2023
Cited by 4 | Viewed by 2046
Abstract
Background: Platelet “Microvesicles” (MVs) are studied for their role in blood coagulation and inflammation. The study aimed to establish if MVs are related to age, plasma levels of inflammation, coagulation, and fibrinolysis markers in healthy individuals. Methods: We prospectively enrolled volunteers aged over [...] Read more.
Background: Platelet “Microvesicles” (MVs) are studied for their role in blood coagulation and inflammation. The study aimed to establish if MVs are related to age, plasma levels of inflammation, coagulation, and fibrinolysis markers in healthy individuals. Methods: We prospectively enrolled volunteers aged over 18 years. MVs, plasma levels of C-reactive protein (CRP), Interleukin 6 (IL-6), Interleukin 10 (IL-10), Interleukin 17 (IL-17), and transforming growth factor β (TGF-β), fibrinogen, plasminogen activator inhibitor-1 (PAI-1), von Willebrand factor (VWF), homocysteine, factor VII (FVII), thrombin activatable fibrinolysis inhibitor (TAFI), and Protein S were tested. Results: A total of 246 individuals (median age 65 years (“IQR”54–72)) were evaluated. Both univariate analysis and logistic regression models showed that MVs positively correlate with age, CRP, IL-6, IL-10, IL-17, TGF-β, fibrinogen, PAI-1, VWF, FVII, and homocysteine, while inversely correlating with TAFI and Protein S. The ROC curve analysis performed to identify a cut off for MV values (700 kMP) showed a good accuracy with over-range cytokines fibrinolysis factor and coagulation markers. Conclusions: To the best of our knowledge, this study is the first to correlate MVs with an entire panel of cardiovascular risk factors in healthy individuals. A future possible role of MVs in screening exams is suggested. Full article
(This article belongs to the Special Issue Personalized Therapy and Clinical Outcomes for Congenital and Acquired Haemorrhagic Disorders, Thromboembolic Disease and Platelet Disorders)
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17 pages, 681 KiB  
Review
Association of microRNA Polymorphisms with Toxicities Induced by Methotrexate in Children with Acute Lymphoblastic Leukemia
by Vasiliki Karpa, Kallirhoe Kalinderi, Liana Fidani and Athanasios Tragiannidis
Hematol. Rep. 2023, 15(4), 634-650; https://doi.org/10.3390/hematolrep15040065 - 20 Nov 2023
Cited by 4 | Viewed by 1965
Abstract
Methotrexate (MTX), a structurally related substance to folic acid, is an important chemotherapeutic agent used for decades in the treatment of pediatric acute lymphoblastic leukemia (ALL) and other types of cancer as non-Hodgkin lymphomas and osteosarcomas. Despite the successful outcomes observed, the primary [...] Read more.
Methotrexate (MTX), a structurally related substance to folic acid, is an important chemotherapeutic agent used for decades in the treatment of pediatric acute lymphoblastic leukemia (ALL) and other types of cancer as non-Hodgkin lymphomas and osteosarcomas. Despite the successful outcomes observed, the primary drawback is the variability in the pharmacokinetics and pharmacodynamics between patients. The main adverse events related to its use are nephrotoxicity, mucositis, and myelosuppression, especially when used in high doses. The potential adverse reactions and toxicities associated with MTX are a cause for concern and may lead to dose reduction or treatment interruption. Genetic variants in MTX transport genes have been linked to toxicity. Pharmacogenetic studies conducted in the past focused on single nucleotide polymorphisms (SNPs) in the coding and 5′-regulatory regions of genes. Recent studies have demonstrated a significant role of microRNAs (miRNAs) in the transport and metabolism of drugs and in the regulation of target genes. In the last few years, the number of annotated miRNAs has continually risen, in addition to the studies of miRNA polymorphisms and MTX toxicity. Therefore, the objective of the present study is to investigate the role of miRNA variants related to MTX adverse effects. Full article
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19 pages, 2791 KiB  
Review
Chimeric Antigen Receptor T Cell Therapy in Acute Myeloid Leukemia: Trials and Tribulations
by Swati Garg, Wei Ni, James D. Griffin and Martin Sattler
Hematol. Rep. 2023, 15(4), 608-626; https://doi.org/10.3390/hematolrep15040063 - 12 Nov 2023
Cited by 3 | Viewed by 3432
Abstract
Acute myeloid leukemia (AML) is a heterogeneous hematological malignancy that is often associated with relapse and drug resistance after standard chemotherapy or targeted therapy, particularly in older patients. Hematopoietic stem cell transplants are looked upon as the ultimate salvage option with curative intent. [...] Read more.
Acute myeloid leukemia (AML) is a heterogeneous hematological malignancy that is often associated with relapse and drug resistance after standard chemotherapy or targeted therapy, particularly in older patients. Hematopoietic stem cell transplants are looked upon as the ultimate salvage option with curative intent. Adoptive cell therapy using chimeric antigen receptors (CAR) has shown promise in B cell malignancies and is now being investigated in AML. Initial clinical trials have been disappointing in AML, and we review current strategies to improve efficacy for CAR approaches. The extensive number of clinical trials targeting different antigens likely reflects the genetic heterogeneity of AML. The limited number of patients reported in multiple early clinical studies makes it difficult to draw conclusions about CAR safety, but it does suggest that the efficacy of this approach in AML lags behind the success observed in B cell malignancies. There is a clear need not only to improve CAR design but also to identify targets in AML that show limited expression in normal myeloid lineage cells. Full article
(This article belongs to the Special Issue Cell Therapies in Hematology: Current Updates and Perspectives)
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12 pages, 1338 KiB  
Article
Changes in Hematological and Hemorheological Parameters Following Mild COVID-19: A 4-Month Follow-Up Study
by Janina Bros, Lars Ibershoff, Emily Zollmann, Jonas Zacher, Fabian Tomschi, Hans-Georg Predel, Wilhelm Bloch and Marijke Grau
Hematol. Rep. 2023, 15(4), 543-554; https://doi.org/10.3390/hematolrep15040057 - 10 Oct 2023
Cited by 4 | Viewed by 2565
Abstract
Background: Coronavirus Disease 2019 (COVID-19) was described to affect red blood cells (RBC) in both severe and mild disease courses. The aim of this study was to investigate whether hematological and hemorheological changes that were previously described for COVID-19 patients after the acute [...] Read more.
Background: Coronavirus Disease 2019 (COVID-19) was described to affect red blood cells (RBC) in both severe and mild disease courses. The aim of this study was to investigate whether hematological and hemorheological changes that were previously described for COVID-19 patients after the acute infection state are still prominent after another 4 months to assess potential long-term effects. Methods: Hematological and RBC rheological parameters, including deformability and aggregation, were measured 41 days after infection in COVID-19 patients and non-COVID control (T0) and 4 months later in COVID-19 patients (T1). Results: The data confirm alterations in hematological parameters, mainly related to cell volume and hemoglobin concentration, but also reduced deformability and increased aggregation at T0 compared to control. While RBC deformability seems to have recovered, hemoglobin-related parameters and RBC aggregation were still impaired at T1. The changes were thus more pronounced in male COVID-19 patients. Conclusion: COVID-19-related changes of the RBC partly consist of several months and might be related to persistent symptoms reported by many COVID-19 patients. Full article
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11 pages, 571 KiB  
Article
Real-World Clinical Characterisation of Polycythaemia Vera Patients from a Prospective Registry in Portugal: Is Resistance to Hydroxyurea a Reality?
by Maria Sarmento, Marta Duarte, Sandra Ponte, Juan Sanchez, Diana Roriz, Laura Fernandes, Maria José Monteiro Silva, Judite Pacheco, Gisela Ferreira, Jorge Freitas, Inês Costa and Daniel Brás
Hematol. Rep. 2023, 15(3), 532-542; https://doi.org/10.3390/hematolrep15030056 - 13 Sep 2023
Cited by 1 | Viewed by 2154
Abstract
Patients with polycythaemia vera (PV) are at increased risk of thrombosis and haemorrhages. Although hydroxyurea (HU) has been the frontline therapy for patients at high risk of vascular complications, about 25% of patients develop resistance/intolerance to this therapy. The aim of this non-interventional, [...] Read more.
Patients with polycythaemia vera (PV) are at increased risk of thrombosis and haemorrhages. Although hydroxyurea (HU) has been the frontline therapy for patients at high risk of vascular complications, about 25% of patients develop resistance/intolerance to this therapy. The aim of this non-interventional, multicentre cohort study was to understand the clinical characteristics and HU treatment response of Portuguese PV patients. HU resistance/intolerance was defined according to adjusted European LeukemiaNet (ELN) criteria. In total, 134 PV patients with a mean (SD) disease duration of 4.8 (5.0) years were included and followed up for 2 years. At baseline, most patients were ≥60 years old (83.2%), at high risk for thrombotic events (87.2%), and receiving HU therapy (79.1%). A total of 10 thrombotic events and 8 haemorrhagic events were reported, resulting in a 5-year probability of thrombo-haemorrhagic events of 17.2%. Haematocrit (p = 0.007), haemoglobin (p = 0.012) and MPN10 symptom score (12.0 (11.6) vs. 10.3 (9.1); p = 0.041) decreased significantly at the 24-month visit compared to baseline. Overall, 75.9% of patients met at least one of the adjusted ELN criteria for HU resistance, and 14.4% of patients remained on HU throughout the study. The results from this real-world study may help identify the subset of patients at higher risk for disease sequelae who may benefit from earlier second-line treatment. Full article
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11 pages, 912 KiB  
Review
How I Manage Chronic Lymphocytic Leukemia
by Patrice Nasnas, Claudio Cerchione, Gerardo Musuraca, Giovanni Martinelli and Alessandra Ferrajoli
Hematol. Rep. 2023, 15(3), 454-464; https://doi.org/10.3390/hematolrep15030047 - 1 Aug 2023
Cited by 6 | Viewed by 4529
Abstract
Chronic lymphocytic leukemia (CLL), is a hematologic malignancy characterized by the uncontrolled proliferation of mature B lymphocytes. CLL is the most prevalent leukemia in Western countries. Its presentation can range from asymptomatic with the incidental finding of absolute lymphocytosis on a routine blood [...] Read more.
Chronic lymphocytic leukemia (CLL), is a hematologic malignancy characterized by the uncontrolled proliferation of mature B lymphocytes. CLL is the most prevalent leukemia in Western countries. Its presentation can range from asymptomatic with the incidental finding of absolute lymphocytosis on a routine blood test, to symptomatic disease requiring immediate intervention. Prognosis of the disease is defined by the presence or absence of specific mutations such as TP53, chromosomal abnormalities such as del(17p), a type of IGHV mutational status, and elevation of B2M and LDH. Treatment of CLL in the United States and Europe has evolved over the recent years thanks to the development of targeted therapies. The standard of care has shifted from traditional chemoimmunotherapy approaches to targeted therapies including Bruton tyrosine kinase inhibitors (BTKis) and BCL2 inhibitors, administered either as monotherapy or in combination with CD20 monoclonal antibodies. Several clinical trials have also recently evaluated combinations of BTKi and venetoclax and showed the combination to be well tolerated and able to induce deep remissions. Targeted therapies have a good safety profile overall; however, they also have unique toxicities that are important to recognize. Diarrhea, fatigue, arthralgia, infections, cytopenias, bleeding, and cardiovascular toxicities (including atrial fibrillation, ventricular arrhythmias, and hypertension) are the adverse events (AEs) commonly associated with BTKis. Initiation of therapy with venetoclax requires close monitoring because of the risk for tumor lysis syndrome associated with this agent, particularly in patients with a high disease burden. Development of newer target therapies is ongoing and the therapeutic landscape in CLL is expanding rapidly. Full article
(This article belongs to the Special Issue State of the Art Papers from 'The Society of Hematologic Oncology Italy – SOHO ITALY')
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7 pages, 422 KiB  
Brief Report
Risk Factors for Death or Cardiovascular Events after Acute Coronary Syndrome in Patients with Myeloproliferative Neoplasms
by Orly Leiva, Andrew Jenkins, Rachel P. Rosovsky, Rebecca K. Leaf, Katayoon Goodarzi and Gabriela Hobbs
Hematol. Rep. 2023, 15(2), 398-404; https://doi.org/10.3390/hematolrep15020040 - 7 Jun 2023
Cited by 6 | Viewed by 3662
Abstract
Patients with myeloproliferative neoplasms (MPNs) are at increased risk of cardiovascular disease (CVD), including acute coronary syndrome (ACS). However, data on long-term outcomes of patients with MPN who have had ACS and risk factors for all-cause death or CV events post-ACS hospitalization are [...] Read more.
Patients with myeloproliferative neoplasms (MPNs) are at increased risk of cardiovascular disease (CVD), including acute coronary syndrome (ACS). However, data on long-term outcomes of patients with MPN who have had ACS and risk factors for all-cause death or CV events post-ACS hospitalization are lacking. We conducted a single-center study of 41 consecutive patients with MPN with ACS hospitalization after MPN diagnosis. After a median follow-up of 80 months after ACS hospitalization, 31 (76%) experienced death or a CV event (myocardial infarction, ischemic stroke, or heart failure hospitalization). After multivariable Cox proportional hazards regression, index ACS within 12 months of MPN diagnosis (HR 3.84, 95% CI 1.44–10.19), WBC ≥ 20 K/µL (HR 9.10, 95% CI 2.71–30.52), JAK2 mutation (HR 3.71, 95% CI 1.22–11.22), and prior CVD (HR 2.60, 95% CI 1.12–6.08) were associated with increased death or CV events. Further studies are warranted to improve cardiovascular outcomes in this patient population. Full article
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12 pages, 923 KiB  
Review
Role of Therapeutic Anticoagulation in COVID-19: The Current Situation
by Mandeep Singh Rahi, Jay Parekh, Prachi Pednekar, Mayuri Mudgal, Vishal Jindal and Kulothungan Gunasekaran
Hematol. Rep. 2023, 15(2), 358-369; https://doi.org/10.3390/hematolrep15020037 - 5 Jun 2023
Cited by 5 | Viewed by 2902
Abstract
Thrombotic complications from COVID-19 are now well known and contribute to significant morbidity and mortality. Different variants confer varying risks of thrombotic complications. Heparin has anti-inflammatory and antiviral effects. Due to its non-anticoagulant effects, escalated-dose anticoagulation, especially therapeutic-dose heparin, has been studied for [...] Read more.
Thrombotic complications from COVID-19 are now well known and contribute to significant morbidity and mortality. Different variants confer varying risks of thrombotic complications. Heparin has anti-inflammatory and antiviral effects. Due to its non-anticoagulant effects, escalated-dose anticoagulation, especially therapeutic-dose heparin, has been studied for thromboprophylaxis in hospitalized patients with COVID-19. Few randomized, controlled trials have examined the role of therapeutic anticoagulation in moderately to severely ill patients with COVID-19. Most of these patients had elevated D-dimers and low bleeding risks. Some trials used an innovative adaptive multiplatform with Bayesian analysis to answer this critical question promptly. All the trials were open-label and had several limitations. Most trials showed improvements in the meaningful clinical outcomes of organ-support-free days and reductions in thrombotic events, mainly in non-critically-ill COVID-19 patients. However, the mortality benefit needed to be more consistent. A recent meta-analysis confirmed the results. Multiple centers initially adopted intermediate-dose thromboprophylaxis, but the studies failed to show meaningful benefits. Given the new evidence, significant societies have suggested therapeutic anticoagulation in carefully selected patients who are moderately ill and do not require an intensive-care-unit level of care. There are multiple ongoing trials globally to further our understanding of therapeutic-dose thromboprophylaxis in hospitalized patients with COVID-19. In this review, we aim to summarize the current evidence regarding the use of anticoagulation in patients with COVID-19 infection. Full article
(This article belongs to the Special Issue Personalized Therapy and Clinical Outcomes for Congenital and Acquired Haemorrhagic Disorders, Thromboembolic Disease and Platelet Disorders)
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11 pages, 277 KiB  
Article
Etiology of Anemia and Risk Factors of Mortality among Hospitalized Patients: A Real-Life Retrospective Study in a Tertiary Center in Greece
by Petros Ioannou, Andria Papazachariou, Maria Tsafaridou, Ioannis E. Koutroubakis and Diamantis P. Kofteridis
Hematol. Rep. 2023, 15(2), 347-357; https://doi.org/10.3390/hematolrep15020036 - 2 Jun 2023
Cited by 1 | Viewed by 2894
Abstract
Anemia is a prominent global health issue with a wide variety of causes and can be associated with decreased quality of life, increased hospitalization, and higher mortality, especially in older individuals. Therefore, studies further shedding light on the causes and the risk factors [...] Read more.
Anemia is a prominent global health issue with a wide variety of causes and can be associated with decreased quality of life, increased hospitalization, and higher mortality, especially in older individuals. Therefore, studies further shedding light on the causes and the risk factors of this condition should be performed. The aim of the present study was to examine the causes of anemia in hospitalized patients in a tertiary hospital in Greece and identify risk factors related to higher mortality. In total, 846 adult patients with a diagnosis of anemia were admitted during the study period. The median age was 81 years, and 44.8% were male. The majority of patients had microcytic anemia, with the median mean corpuscular volume (MCV) being 76.3 fL and the median hemoglobin being 7.1 g/dL. Antiplatelets were used by 28.6% of patients, while 28.4% were using anticoagulants at the time of diagnosis. At least one unit of packed red blood cells (PRBCs) was transfused in 84.6% of patients, and a median of two PRBCs was used per patient. A gastroscopy was performed in 55%, and a colonoscopy was performed in 39.8% of patients in the present cohort. Anemia was considered to be multifactorial in almost half the cases, while the most commonly identified cause was iron deficiency anemia, more commonly with positive endoscopic findings. Mortality was relatively low, at 4.1%. Multivariate logistic regression analysis identified higher B12 levels and longer duration of hospital stay to be independently positively associated with mortality. Full article
16 pages, 1443 KiB  
Review
Combination Therapies with Kinase Inhibitors for Acute Myeloid Leukemia Treatment
by Shinichiro Takahashi
Hematol. Rep. 2023, 15(2), 331-346; https://doi.org/10.3390/hematolrep15020035 - 24 May 2023
Cited by 9 | Viewed by 3080
Abstract
Targeting kinase activity is considered to be an attractive therapeutic strategy to overcome acute myeloid leukemia (AML) since aberrant activation of the kinase pathway plays a pivotal role in leukemogenesis through abnormal cell proliferation and differentiation block. Although clinical trials for kinase modulators [...] Read more.
Targeting kinase activity is considered to be an attractive therapeutic strategy to overcome acute myeloid leukemia (AML) since aberrant activation of the kinase pathway plays a pivotal role in leukemogenesis through abnormal cell proliferation and differentiation block. Although clinical trials for kinase modulators as single agents remain scarce, combination therapies are an area of therapeutic interest. In this review, the author summarizes attractive kinase pathways for therapeutic targets and the combination strategies for these pathways. Specifically, the review focuses on combination therapies targeting the FLT3 pathways, as well as PI3K/AKT/mTOR, CDK and CHK1 pathways. From a literature review, combination therapies with the kinase inhibitors appear more promising than monotherapies with individual agents. Therefore, the development of efficient combination therapies with kinase inhibitors may result in effective therapeutic strategies for AML. Full article
(This article belongs to the Topic Myeloma and Leukemia-Challenges and Current Treatment Options)
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7 pages, 661 KiB  
Case Report
A Report of a Symptomatic Progressive Myeloma during Pregnancy and Postpartum Period from Asymptomatic State
by Gehad Elgabry, Lydia Spencer, Hisam Siddiqi, Soumya Ojha and Farooq Wandroo
Hematol. Rep. 2023, 15(2), 305-311; https://doi.org/10.3390/hematolrep15020031 - 5 May 2023
Cited by 2 | Viewed by 2537
Abstract
 Multiple myeloma is a plasma cell malignancy that is most commonly observed in males in the sixth and seventh decade of life. The clinical scenario of multiple myeloma with concurrent pregnancy is considered to be very rare. We detail here the case [...] Read more.
 Multiple myeloma is a plasma cell malignancy that is most commonly observed in males in the sixth and seventh decade of life. The clinical scenario of multiple myeloma with concurrent pregnancy is considered to be very rare. We detail here the case of a young female with known IgG kappa multiple myeloma who was found to have a steady elevation of her IgG kappa paraprotein during pregnancy and symptomatic progression in the postpartum period. She delivered a healthy baby at 40 weeks gestation. We present a review of all reported cases of known multiple myeloma progressing during pregnancy and in the postpartum period, the treatments given, and their outcomes. The report also provides suggestions for diagnosis and management of myeloma during pregnancy in order to have an outcome of successful uncomplicated pregnancy with healthy offspring.  Full article
(This article belongs to the Special Issue Opportunities and Challenges in Multiple Myeloma Treatment and Management)
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8 pages, 2063 KiB  
Case Report
Testosterone Usage Leading to Pulmonary Embolisms and Deep Vein Thrombosis: A Case Report and Review of the Literature
by Sasmith R. Menakuru, Mona Atta, Vijaypal S. Dhillon and Ahmed Salih
Hematol. Rep. 2023, 15(2), 290-297; https://doi.org/10.3390/hematolrep15020029 - 26 Apr 2023
Cited by 5 | Viewed by 4274
Abstract
Androgen usage has widely increased in recent times via prescribed and unprescribed means. Testosterone is a popular androgen taken by both athletes and the general population. While there is some evidence of androgens being thrombogenic, we report on a 19-year-old male who presented [...] Read more.
Androgen usage has widely increased in recent times via prescribed and unprescribed means. Testosterone is a popular androgen taken by both athletes and the general population. While there is some evidence of androgens being thrombogenic, we report on a 19-year-old male who presented to the hospital after the usage of testosterone for one month, leading to the development of multiple pulmonary emboli and deep vein thrombosis. The authors hope to elucidate the relationship between testosterone usage and thrombosis formation. Full article
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7 pages, 577 KiB  
Case Report
A Case of Vancomycin-Induced Severe Immune Thrombocytopenia
by Shivani Shah, Ryan Sweeney, Maitreyee Rai and Deep Shah
Hematol. Rep. 2023, 15(2), 283-289; https://doi.org/10.3390/hematolrep15020028 - 24 Apr 2023
Cited by 3 | Viewed by 3752
Abstract
A male in his 60s presented with left lower extremity fractures following a vehicle accident. Hemoglobin, initially, was 12.4 mmol/L, and platelet count was 235 k/mcl. On day 11 of admission, his platelet count initially dropped to 99 k/mcl, and after recovery it [...] Read more.
A male in his 60s presented with left lower extremity fractures following a vehicle accident. Hemoglobin, initially, was 12.4 mmol/L, and platelet count was 235 k/mcl. On day 11 of admission, his platelet count initially dropped to 99 k/mcl, and after recovery it rapidly decreased to 11 k/mcl on day 16 when the INR was 1.3 and aPTT was 32 s, and he continued to have a stable anemia throughout admission. There was no response in platelet count post-transfusion of four units of platelets. Hematology initially evaluated the patient for disseminated intravascular coagulation, heparin-induced thrombocytopenia (anti-PF4 antibody was 0.19), and thrombotic thrombocytopenic purpura (PLASMIC score of 4). Vancomycin was administered on days 1–7 for broad spectrum antimicrobial coverage and day 10, again, for concerns of sepsis. Given the temporal association of thrombocytopenia and vancomycin administration, a diagnosis of vancomycin-induced immune thrombocytopenia was established. Vancomycin was discontinued, and 2 doses of 1000 mg/kg of intravenous immunoglobulin 24 h apart were administered with the subsequent resolution of thrombocytopenia. Full article
(This article belongs to the Special Issue Personalized Therapy and Clinical Outcomes for Congenital and Acquired Haemorrhagic Disorders, Thromboembolic Disease and Platelet Disorders)
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17 pages, 1550 KiB  
Article
Changes in Hematologic Lab Measures Observed in Patients with Paroxysmal Nocturnal Hemoglobinuria Treated with C5 Inhibitors, Ravulizumab and Eculizumab: Real-World Evidence from a US Based EMR Network
by Jesse Fishman, Seth Kuranz, Michael M. Yeh, Kaylen Brzozowski and Herman Chen
Hematol. Rep. 2023, 15(2), 266-282; https://doi.org/10.3390/hematolrep15020027 - 21 Apr 2023
Cited by 8 | Viewed by 3839
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH), a rare acquired hematologic disorder, can be treated with C5 inhibitors (C5i) such as eculizumab or ravulizumab. This retrospective study is the first to describe real-world treatment patterns and changes in hematologic PNH-monitoring laboratory tests among C5i-treated US patients. [...] Read more.
Paroxysmal nocturnal hemoglobinuria (PNH), a rare acquired hematologic disorder, can be treated with C5 inhibitors (C5i) such as eculizumab or ravulizumab. This retrospective study is the first to describe real-world treatment patterns and changes in hematologic PNH-monitoring laboratory tests among C5i-treated US patients. Data were extracted from TriNetX Dataworks Network and included patients with a PNH diagnosis between 1 January 2010, and 20 August 2021. Patients were stratified into three cohorts based on their C5i usage: eculizumab, ravulizumab (prior eculizumab), and ravulizumab (eculizumab naïve). Hematological markers (hemoglobin [Hb], lactate dehydrogenase [LDH], and absolute reticulocyte count [ARC]) and relevant clinical events (e.g., breakthrough hemolysis [BTH], complement-amplifying conditions [CAC], thrombosis, infection, and all-cause mortality) were captured any time within 12 months post-index treatment. Of the 143 (eculizumab), 43 (ravulizumab, prior eculizumab), and 33 (ravulizumab, eculizumab naïve) patients, mean age across cohorts was 42–51 years, 55–61% were female, 63–73% were White, and 33–40% had aplastic anemia. Among all cohorts 12 months post-C5i treatment, 50–82% remained anemic, 8–32% required ≥1 transfusion, and 13–59% had BTH, of which 33%-54% had CACs. Additionally, thrombosis was seen in 7–15% of patients, infection in 20–25%, and mortality in 1–7%. These findings suggest many C5i-treated patients experience suboptimal disease control. Full article
(This article belongs to the Special Issue Personalized Therapy and Clinical Outcomes for Congenital and Acquired Haemorrhagic Disorders, Thromboembolic Disease and Platelet Disorders)
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12 pages, 1595 KiB  
Article
Molecular Tumor Boards: The Next Step towards Precision Therapy in Cancer Care
by Angela Liu, Paige Vicenzi, Ishna Sharma, Kaci Orr, Christa Teller, Micha Koentz, Heidi Trinkman, Kelly Vallance and Anish Ray
Hematol. Rep. 2023, 15(2), 244-255; https://doi.org/10.3390/hematolrep15020025 - 4 Apr 2023
Cited by 8 | Viewed by 4053
Abstract
The application of molecular tumor profiles in clinical decision making remains a challenge. To aid in the interpretation of complex biomarkers, molecular tumor boards (MTBs) have been established worldwide. In the present study, we show that a multidisciplinary approach is essential to the [...] Read more.
The application of molecular tumor profiles in clinical decision making remains a challenge. To aid in the interpretation of complex biomarkers, molecular tumor boards (MTBs) have been established worldwide. In the present study, we show that a multidisciplinary approach is essential to the success of MTBs. Our MTB, consisting of pediatric oncologists, pathologists, and pharmacists, evaluated 115 cases diagnosed between March 2016 and September 2021. If targetable mutations were identified, pharmacists aided in the evaluation of treatment options based on drug accessibility. Treatable genetic alterations detected through molecular testing most frequently involved the cell cycle. For 85% of the cases evaluated, our MTB provided treatment recommendations based on the patient’s history and results of molecular tumor testing. Only three patients, however, received MTB-recommended targeted therapy, and only one of these patients demonstrated an improved clinical outcome. For the remaining patients, MTB-recommended treatment often was not administered because molecular tumor profiling was not performed until late in the disease course. For the three patients who did receive MTB-recommended therapy, such treatment was not administered until months after diagnosis due to physician preference. Thus, the education of healthcare providers regarding the benefits of targeted therapy may increase acceptance of these novel agents and subsequently improve patient survival. Full article
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13 pages, 4342 KiB  
Case Report
Mutational Profile and Pathological Features of a Case of Interleukin-10 and RGS1-Positive Spindle Cell Variant Diffuse Large B-Cell Lymphoma
by Joaquim Carreras, Yara Yukie Kikuti, Masashi Miyaoka, Shinichiro Hiraiwa, Sakura Tomita, Haruka Ikoma, Yusuke Kondo, Atsushi Ito, Shunsuke Nagase, Hisanobu Miura, Giovanna Roncador, Lluis Colomo, Rifat Hamoudi, Elias Campo and Naoya Nakamura
Hematol. Rep. 2023, 15(1), 188-200; https://doi.org/10.3390/hematolrep15010020 - 12 Mar 2023
Cited by 3 | Viewed by 3685
Abstract
Diffuse large B-cell lymphoma with spindle cell morphology is a rare variant. We present the case of a 74-year-old male who initially presented with a right supraclavicular (lymph) node enlargement. Histological analysis showed a proliferation of spindle-shaped cells with narrow cytoplasms. An immunohistochemical [...] Read more.
Diffuse large B-cell lymphoma with spindle cell morphology is a rare variant. We present the case of a 74-year-old male who initially presented with a right supraclavicular (lymph) node enlargement. Histological analysis showed a proliferation of spindle-shaped cells with narrow cytoplasms. An immunohistochemical panel was used to exclude other tumors, such as melanoma, carcinoma, and sarcoma. The lymphoma was characterized by a cell-of-origin subtype of germinal center B-cell-like (GCB) based on Hans’ classifier (CD10-negative, BCL6-positive, and MUM1-negative); EBER negativity, and the absence of BCL2, BCL6, and MYC rearrangements. Mutational profiling using a custom panel of 168 genes associated with aggressive B-cell lymphomas confirmed mutations in ACTB, ARID1B, DUSP2, DTX1, HLA-B, PTEN, and TNFRSF14. Based on the LymphGen 1.0 classification tool, this case had an ST2 subtype prediction. The immune microenvironment was characterized by moderate infiltration of M2-like tumor-associated macrophages (TMAs) with positivity of CD163, CSF1R, CD85A (LILRB3), and PD-L1; moderate PD-1 positive T cells, and low FOXP3 regulatory T lymphocytes (Tregs). Immunohistochemical expression of PTX3 and TNFRSF14 was absent. Interestingly, the lymphoma cells were positive for HLA-DP-DR, IL-10, and RGS1, which are markers associated with poor prognosis in DLBCL. The patient was treated with R-CHOP therapy, and achieved a metabolically complete response. Full article
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6 pages, 512 KiB  
Brief Report
Frail Multiple Myeloma Patients Deserve More Than Just a Score
by Hannah Louise Miller and Faye Amelia Sharpley
Hematol. Rep. 2023, 15(1), 151-156; https://doi.org/10.3390/hematolrep15010015 - 21 Feb 2023
Cited by 3 | Viewed by 3380
Abstract
Frailty is a hot topic in the field of multiple myeloma (MM). Clinicians have realised that frail myeloma patients can struggle with treatment, resulting in dose reductions and treatment discontinuation, which risk shorter progression-free and overall survival. Efforts have focused on the validity [...] Read more.
Frailty is a hot topic in the field of multiple myeloma (MM). Clinicians have realised that frail myeloma patients can struggle with treatment, resulting in dose reductions and treatment discontinuation, which risk shorter progression-free and overall survival. Efforts have focused on the validity of existing frailty scores and on the development of new indices to identify frail patients more accurately. This review article explores the challenges of the existing frailty scores, including the International Myeloma Working Group (IMWG) frailty score, the revised Myeloma Co-morbidity Index (R-MCI), and the Myeloma Risk Profile (MRP). We conclude that the missing link is for frailty scoring to translate into a tool useful in real-world clinical practice. The future of frailty scores lies in their ability to be woven into clinical trials, to create a robust clinical evidence base for treatment selection and dose modification, and also to identify a cohort of patients who merit additional support from the wider MM multidisciplinary team. Full article
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11 pages, 2145 KiB  
Article
Post-Treatment Neutrophil and Lymphocyte Counts Predict Progression-Free Survival Following First-Line Chemotherapy in Hodgkin’s Lymphoma
by Grace Fangmin Tan, Siting Goh, Esther Wei Yin Chang, Ya Hwee Tan, Jianbang Chiang, Valerie Shiwen Yang, Eileen Yi Ling Poon, Nagavalli Somasundaram, Mohamad Farid Bin Harunal Rashid, Miriam Tao, Soon Thye Lim, Choon Kiat Ong and Jason Yongsheng Chan
Hematol. Rep. 2023, 15(1), 108-118; https://doi.org/10.3390/hematolrep15010012 - 10 Feb 2023
Cited by 1 | Viewed by 3133
Abstract
Hodgkin’s lymphoma carries an excellent prognosis with modern chemotherapy, but a significant proportion of patients remain refractory to or relapse after first-line treatment. Immunological changes post-treatment, such as chemotherapy-induced neutropenia (CIN) or lymphopenia, have shown prognostic significance in multiple tumor types. Our study [...] Read more.
Hodgkin’s lymphoma carries an excellent prognosis with modern chemotherapy, but a significant proportion of patients remain refractory to or relapse after first-line treatment. Immunological changes post-treatment, such as chemotherapy-induced neutropenia (CIN) or lymphopenia, have shown prognostic significance in multiple tumor types. Our study aims to investigate the prognostic value of immunologic changes in Hodgkin’s lymphoma by examining the post-treatment lymphocyte count (pALC), neutrophil count (pANC) and the neutrophil-lymphocyte ratio (pNLR). Patients treated for classical Hodgkin’s lymphoma at the National Cancer Centre Singapore using ABVD-based regimens were retrospectively analyzed. An optimal cut-off value for high pANC, low pALC and high pNLR in predicting progression-free survival was determined by receiver operating curve analysis. Survival analysis was performed using the Kaplan–Meier method and multivariable Cox proportional models. Overall OS and PFS were excellent, with a 5-year OS of 99.2% and a 5-year PFS of 88.2%. Poorer PFS was associated with high pANC (HR 2.99, p = 0.0392), low pALC (HR 3.95, p = 0.0038) and high pNLR (p = 0.0078). In conclusion, high pANC, low pALC and high pNLR confer a poorer prognosis for Hodgkin’s lymphoma. Future studies should evaluate the potential of improving treatment outcomes by the adjustment of chemotherapy dose intensity based on post-treatment blood counts. Full article
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10 pages, 2115 KiB  
Article
Synergistic Interactions between the Hypomethylating Agent Thio-Deoxycytidine and Venetoclax in Myelodysplastic Syndrome Cells
by Xiaoyan Hu, Lin Li, Jewel Nkwocha, Kanika Sharma, Liang Zhou and Steven Grant
Hematol. Rep. 2023, 15(1), 91-100; https://doi.org/10.3390/hematolrep15010010 - 2 Feb 2023
Cited by 5 | Viewed by 2374
Abstract
Interactions between the novel hypomethylating agent (HMA) thio-deoxycytidine (T-dCyd) and the BCL-2 antagonist ABT-199 (venetoclax) have been examined in human myelodysplastic syndrome (MDS) cells. The cells were exposed to agents alone or in combination, after which apoptosis was assessed, and a Western blot [...] Read more.
Interactions between the novel hypomethylating agent (HMA) thio-deoxycytidine (T-dCyd) and the BCL-2 antagonist ABT-199 (venetoclax) have been examined in human myelodysplastic syndrome (MDS) cells. The cells were exposed to agents alone or in combination, after which apoptosis was assessed, and a Western blot analysis was performed. Co-administration of T-dCyd and ABT-199 was associated with the down-regulation of DNA methyltransferase 1 (DNMT1) and synergistic interactions documented by a Median Dose Effect analysis in multiple MDS-derived lines (e.g., MOLM-13, SKM-1, and F-36P). Inducible BCL-2 knock-down significantly increased T-dCyd’s lethality in MOLM-13 cells. Similar interactions were observed in the primary MDS cells, but not in the normal cord blood CD34+ cells. Enhanced killing by the T-dCyd/ABT-199 regimen was associated with increased reactive oxygen species (ROS) generation and the down-regulation of the anti-oxidant proteins Nrf2 and HO-1, as well as BCL-2. Moreover, ROS scavengers (e.g., NAC) reduced lethality. Collectively, these data suggest that combining T-dCyd with ABT-199 kills MDS cells through an ROS-dependent mechanism, and we argue that this strategy warrants consideration in MDS therapy. Full article
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15 pages, 20447 KiB  
Review
JAK2 Mutations Are Rare and Diverse in Myelodysplastic Syndromes: Case Series and Review of the Literature
by Melissa Delio, Christine Bryke, Lourdes Mendez, Loren Joseph and Sarmad Jassim
Hematol. Rep. 2023, 15(1), 73-87; https://doi.org/10.3390/hematolrep15010008 - 18 Jan 2023
Cited by 3 | Viewed by 4490
Abstract
Objectives: To investigate and characterize JAK2 mutations in myelodysplastic syndrome (MDS), we present three cases with diverse JAK2 mutations and review the literature. Methods: The institutional SoftPath software was used to find MDS cases between January 2020 and April 2022. The cases with [...] Read more.
Objectives: To investigate and characterize JAK2 mutations in myelodysplastic syndrome (MDS), we present three cases with diverse JAK2 mutations and review the literature. Methods: The institutional SoftPath software was used to find MDS cases between January 2020 and April 2022. The cases with a diagnosis of a myelodysplastic/myeloproliferative overlap syndrome including MDS/MPN with ring sideroblasts and thrombocytosis were excluded. The cases with molecular data by next generation sequencing looking for gene aberrations commonly seen in myeloid neoplasms were reviewed for the detection of JAK2 mutations including variants. A literature review on the identification, characterization, and significance of JAK2 mutations in MDS was performed. Results: Among 107 cases of the MDS reviewed, a JAK2 mutation was present in three cases, representing 2.8% of the overall cases. A JAK2 V617F mutation was found in one case representing slightly less than 1% of all the MDS cases. In addition, we found JAK2 R564L and JAK2 I670V point mutation variants to be associated with a myelodysplastic phenotype. Conclusions: JAK2 mutations in MDS are rare and represent less than 3% of cases. It appears that JAK2 variant mutations in MDS are diverse and further studies are needed to understand their role in the phenotype and prognosis of the disease. Full article
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27 pages, 1377 KiB  
Review
Monoclonal Gammopathies and the Bone Marrow Microenvironment: From Bench to Bedside and Then Back Again
by Federica Plano, Anna Maria Corsale, Emilia Gigliotta, Giulia Camarda, Candida Vullo, Marta Di Simone, Mojtaba Shekarkar Azgomi, Maria Speciale, Melania Carlisi, Nadia Caccamo, Francesco Dieli, Serena Meraviglia, Sergio Siragusa and Cirino Botta
Hematol. Rep. 2023, 15(1), 23-49; https://doi.org/10.3390/hematolrep15010004 - 9 Jan 2023
Cited by 4 | Viewed by 4413
Abstract
Multiple myeloma (MM) is an incurable hematologic malignancy characterized by a multistep evolutionary pathway, with an initial phase called monoclonal gammopathy of undetermined significance (MGUS), potentially evolving into the symptomatic disease, often preceded by an intermediate phase called “smoldering” MM (sMM). From a [...] Read more.
Multiple myeloma (MM) is an incurable hematologic malignancy characterized by a multistep evolutionary pathway, with an initial phase called monoclonal gammopathy of undetermined significance (MGUS), potentially evolving into the symptomatic disease, often preceded by an intermediate phase called “smoldering” MM (sMM). From a biological point of view, genomic alterations (translocations/deletions/mutations) are already present at the MGUS phase, thus rendering their role in disease evolution questionable. On the other hand, we currently know that changes in the bone marrow microenvironment (TME) could play a key role in MM evolution through a progressive shift towards a pro-inflammatory and immunosuppressive shape, which may drive cancer progression as well as clonal plasma cells migration, proliferation, survival, and drug resistance. Along this line, the major advancement in MM patients’ survival has been achieved by the introduction of microenvironment-oriented drugs (including immunomodulatory drugs and monoclonal antibodies). In this review, we summarized the role of the different components of the TME in MM evolution from MGUS as well as potential novel therapeutic targets/opportunities. Full article
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7 pages, 1156 KiB  
Article
Cutaquig® Is Well Tolerated in Immunodeficient Patients Who Did Not Tolerate Other Subcutaneous Immunoglobulin Products
by Sydney Brownlee, Crystal Allen, Mohammed F. Kana’an, D. William Cameron and Juthaporn Cowan
Hematol. Rep. 2022, 14(4), 342-348; https://doi.org/10.3390/hematolrep14040048 - 17 Nov 2022
Cited by 1 | Viewed by 2209
Abstract
Objective: Subcutaneous immunoglobulin (SCIG) treatment is generally tolerable, but some patients may experience adverse events to one or more SCIG products. We investigated whether 16.5% Cutaquig® treatment offered a tolerable and safe alternative treatment for immunodeficient patients. Methods: A one-year prospective cohort [...] Read more.
Objective: Subcutaneous immunoglobulin (SCIG) treatment is generally tolerable, but some patients may experience adverse events to one or more SCIG products. We investigated whether 16.5% Cutaquig® treatment offered a tolerable and safe alternative treatment for immunodeficient patients. Methods: A one-year prospective cohort study was conducted at a single center in Ottawa, Canada. Adult immunodeficient patients who reported previous intolerability, adverse events, or other difficulty to other 20% SCIG product(s) were recruited to start on 16.5% Cutaquig®. Treatment tolerability, safety, and quality of life were observed and described. Results: Seven out of ten patients tolerated Cutaquig®. There were no serious or severe adverse events related to the treatment. Three moderate infections were reported (two urinary tract infections and one injection site infection). The mean serum IgG level at the end of the study was comparable to baseline levels recorded before the study: 9.6 ± 4.5 vs. 7.6 ± 4.3 g/L, p = 0.07. The overall health and health domain changes in the SF-36 and quality of life tests using the EQ visual analog scale improved by 21.5% (p = 0.38), 16.7% (p = 0.29), and 7.7% (p = 0.23), respectively. Conclusions: Cutaquig® may be used as an alternative treatment option for patients who did not tolerate 20% SCIG products. Full article
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12 pages, 1633 KiB  
Case Report
Acute Promyelocytic Leukemia in a Woman with Thalassemia Intermedia: Case Report and Review of Literature on Hematological Malignancies in β-Thalassemia Patients
by Claudio Pellegrino, Giulia Dragonetti, Patrizia Chiusolo, Monica Rossi, Nicoletta Orlando and Luciana Teofili
Hematol. Rep. 2022, 14(4), 310-321; https://doi.org/10.3390/hematolrep14040045 - 21 Oct 2022
Cited by 4 | Viewed by 7162
Abstract
Patients affected by transfusion-dependent β-thalassemia are prone to developing several clinical complications, mostly related to the iron overload. We report the case of a patient affected by transfusion-dependent β-thalassemia (TDT) developing acute promyelocytic leukemia (APL). In our case, the therapeutic management was significantly [...] Read more.
Patients affected by transfusion-dependent β-thalassemia are prone to developing several clinical complications, mostly related to the iron overload. We report the case of a patient affected by transfusion-dependent β-thalassemia (TDT) developing acute promyelocytic leukemia (APL). In our case, the therapeutic management was significantly complicated not only by myocardial dysfunction, but also by the occurrence of the differentiation syndrome following all-trans retinoic acid (ATRA) administration. We carried out a careful revision of the current literature on the occurrence of hematological malignancies in β-thalassemia patients to investigate the major complications so far described. APL occurrence in β-thalassemia patients has been very rarely reported, and our experience suggests that TDT patients suffering pre-existing comorbidities may develop a potentially fatal complication during ATRA therapy. Full article
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10 pages, 588 KiB  
Article
Bisphosphonate Use for Glucocorticoid-Induced Osteoporosis in Elderly Patients with Immune Thrombocytopenia Receiving Prolonged Steroid Therapy: A Single Institute Retrospective Study
by Satoshi Yamasaki, Kenjiro Kamezaki, Yoshikiyo Ito and Takahiko Horiuchi
Hematol. Rep. 2022, 14(3), 276-285; https://doi.org/10.3390/hematolrep14030039 - 19 Sep 2022
Cited by 2 | Viewed by 2814
Abstract
Prednisolone, used as a standard initial treatment for immune thrombocytopenia (ITP), is an important risk factor for osteoporosis. To investigate the prevention of glucocorticoid-induced osteoporosis (GIO) in elderly ITP patients receiving prolonged steroid therapy, associations between GIO prevention and the real-world data of [...] Read more.
Prednisolone, used as a standard initial treatment for immune thrombocytopenia (ITP), is an important risk factor for osteoporosis. To investigate the prevention of glucocorticoid-induced osteoporosis (GIO) in elderly ITP patients receiving prolonged steroid therapy, associations between GIO prevention and the real-world data of score changes of a dual-energy X-ray absorptiometry (DXA) scan, FRAX® and the Garvan tool during the initial loading of prednisolone were examined. In our institute, 22 ITP patients aged ≥ 70 years received 0.5–1.0 mg/kg prednisolone for 2–3 weeks as the initial ITP treatment between 2014 and 2021. The femoral neck bone mineral density (BMD) measured by DXA scan was entered into FRAX® to define the risk-adapted approach to bisphosphonate during the initial loading of prednisolone. Bisphosphonate was administered according to <−1.0 femoral neck BMD T-score measured by DXA scan. Worse scores of FRAX® and the Garvan tool were associated with bisphosphonate use for short-term fracture prevention in primary GIO; however, there were no incidents of fracture or significant differences in probabilities determined by FRAX® and the Garvan tool. During the initial loading of prednisolone, prescribing bisphosphonate might prevent the reduction in BMD in elderly patients with ITP receiving prolonged steroid therapy. Full article
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4 pages, 425 KiB  
Case Report
Successful Treatment of Autoimmune Hemolytic Anemia Concomitant with Proliferation of Epstein-Barr Virus in a Post-Heart Transplant Patient
by Dan Ran Castillo, Parthiv Sheth, Kevin Nishino, Wesley Tait Stevens, Anthony Nguyen, Alberto Romagnolo and Hamid Mirshahidi
Hematol. Rep. 2022, 14(3), 261-264; https://doi.org/10.3390/hematolrep14030036 - 17 Aug 2022
Cited by 3 | Viewed by 3079
Abstract
Autoimmune hemolytic anemia (AIHA) is a rare complication following heart transplantation and has been attributed to several etiologies including infections, immunosuppressive medications, and post-transplant lymphoproliferative disorders. We report a 23-year-old male presenting 22 years after heart transplantation with severe AIHA. Laboratory findings were [...] Read more.
Autoimmune hemolytic anemia (AIHA) is a rare complication following heart transplantation and has been attributed to several etiologies including infections, immunosuppressive medications, and post-transplant lymphoproliferative disorders. We report a 23-year-old male presenting 22 years after heart transplantation with severe AIHA. Laboratory findings were notable for positive IgG autoantibody against RBCs and high titer Epstein-Barr virus (EBV) viremia. Shortly after the first unit of irradiated RBC transfusion and high dose steroids, the patient developed acute dyspnea and hypoxia requiring intubation. Further workup demonstrated that the patient had Methicillin-sensitive Staphylococcus aureus (MSSA) pneumonia (PNA) and bacteremia, requiring antibiotics. Patient was subsequently treated with high-dose steroids, IVIG, as well as rituximab. Following treatment, the patient was successfully extubated and eventually showed complete resolution of the anemia. This case is novel as it represents AIHA likely secondary to EBV viremia in a post-cardiac transplant patient complicated by a severe transfusion reaction. In this circumstance, rituximab in conjunction with standard of care remains an effective treatment of choice. Full article
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8 pages, 526 KiB  
Review
COVID-19-Associated Thrombotic Thrombocytopenic Purpura: A Case Report and Systematic Review
by Haseeb Chaudhary, Usama Nasir, Khezar Syed, Maria Labra, Christopher Reggio, Ansar Aziz, Parin Shah, Roopika Reddy and Navdeep Sangha
Hematol. Rep. 2022, 14(3), 253-260; https://doi.org/10.3390/hematolrep14030035 - 2 Aug 2022
Cited by 13 | Viewed by 3465
Abstract
Introduction: The proliferation of literature regarding the COVID-19 pandemic has served to highlight a wide spectrum of disease manifestations and complications, such as thrombotic microangiopathies. Our review with a brief case presentation highlights the increasing recognition of TTP in COVID-19 and describes its [...] Read more.
Introduction: The proliferation of literature regarding the COVID-19 pandemic has served to highlight a wide spectrum of disease manifestations and complications, such as thrombotic microangiopathies. Our review with a brief case presentation highlights the increasing recognition of TTP in COVID-19 and describes its salient characteristics. Methods: We screened the available literature in PubMed, EMBASE, and Cochrane databases from inception until April 2022 of articles mentioning COVID-19-associated TTP in English language. Results: From 404 records, we included 8 articles mentioning data of 11 patients in our review. TTP was predominantly reported in females (72%) with a mean age of 48.2 years (SD 15.1). Dyspnea was the most common symptom in one third of patients (36.6%). Neurological symptoms were reported in 27.3% of cases. The time to diagnosis of TTP was 10 days (SD 5.8) from onset of COVID-19. All 11 cases underwent plasma exchange (PLEX), with a mean of 12 sessions per patient, whereas 6 cases received Rituximab (54.5%), and 3 received Caplacizumab (27.3%). One patient died from the illness. Conclusion: This review of available literature highlights the atypical and refractory nature of COVID-19-associated TTP. It required longer sessions of PLEX, with half of the patients receiving at least one immunosuppressant. Full article
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5 pages, 1209 KiB  
Case Report
Efficacy of Eltrombopag in Children with Post-Stem Cell Transplant Prolonged Isolated Thrombocytopenia
by Megumi Matsumoto, Kazuki Terada, Taichiro Tsuchimochi, Satoko Takahashi, Yasushi Noguchi and Shunji Igarashi
Hematol. Rep. 2022, 14(3), 240-244; https://doi.org/10.3390/hematolrep14030033 - 1 Aug 2022
Cited by 2 | Viewed by 2405
Abstract
Prolonged isolated thrombocytopenia (PIT) is a complication following allogeneic hematopoietic cell transplantation that results in prolonged transfusion dependence. Recently, the efficacy of a thrombopoietin receptor agonist (eltrombopag) against PIT has been reported in adults; however, there are few reports in children. A 4-year-old [...] Read more.
Prolonged isolated thrombocytopenia (PIT) is a complication following allogeneic hematopoietic cell transplantation that results in prolonged transfusion dependence. Recently, the efficacy of a thrombopoietin receptor agonist (eltrombopag) against PIT has been reported in adults; however, there are few reports in children. A 4-year-old male pediatric patient diagnosed with congenital pure red cell aplasia underwent allogeneic hematopoietic cell transplantation. Neutrophil engraftment was observed on post-transplant Day 26; however, platelet counts remained <10 × 109/L. Transfusions were required 1–2 times a week for at least 4 months. On post-transplant Day 124, oral eltrombopag (up to 2.4 mg/kg/day) was initiated. Thereafter, the platelet counts were maintained at ≥10 × 109/L, and the patient became transfusion independent. At 2 years and 6 months after the oral administration, no chromosomal abnormalities, thromboembolism, or myelofibrosis was observed. Thus, eltrombopag can be a potential treatment option for pediatric PIT. Full article
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5 pages, 228 KiB  
Case Report
Acute Hyperhemolysis Syndrome in a Patient with Known Sickle Cell Anemia Refractory to Steroids and IVIG Treated with Tocilizumab and Erythropoietin: A Case Report and Review of Literature
by Sasmith R. Menakuru, Adelina Priscu, Vijaypal Dhillon and Ahmed Salih
Hematol. Rep. 2022, 14(3), 235-239; https://doi.org/10.3390/hematolrep14030032 - 21 Jul 2022
Cited by 13 | Viewed by 3891
Abstract
Patients with sickle cell anemia often receive multiple red blood cell (RBC) transfusions during their lifetime. Hyperhemolysis is a life-threatening phenomenon of accelerated hemolysis and worsening anemia that occurs when both transfused RBCs and autologous RBCs are destroyed. The level of hemoglobin post-transfusion [...] Read more.
Patients with sickle cell anemia often receive multiple red blood cell (RBC) transfusions during their lifetime. Hyperhemolysis is a life-threatening phenomenon of accelerated hemolysis and worsening anemia that occurs when both transfused RBCs and autologous RBCs are destroyed. The level of hemoglobin post-transfusion is lower than pre-transfusion levels, and patients are usually hemodynamically unstable. Hyperhemolysis must be differentiated from a delayed hemolytic transfusion reaction during which destruction of transfused RBC is the cause of anemia. Hyperhemolysis syndrome can be differentiated into acute (within seven days) and chronic forms (after seven days) post-transfusion. The authors present a case of acute hyperhemolysis syndrome in a patient with sickle cell anemia refractory to steroids and IVIG, which are the treatment of choice. The patient was treated with tocilizumab, combined with supportive measures of erythropoietin, iron, vitamin B12, and folate. Full article
3 pages, 198 KiB  
Case Report
Severe Hemolytic Anemia due to Vitamin B12 Deficiency in Six Months
by Narayanan Sadagopan
Hematol. Rep. 2022, 14(3), 210-212; https://doi.org/10.3390/hematolrep14030028 - 22 Jun 2022
Cited by 5 | Viewed by 3493
Abstract
Gastric bypass is a common cause of vitamin B12 deficiency. It can lead to patients presenting with symptoms of anemia. The body has significant reserves of vitamin B12 and loses vitamin B12 slowly. The following case is of a patient who underwent a [...] Read more.
Gastric bypass is a common cause of vitamin B12 deficiency. It can lead to patients presenting with symptoms of anemia. The body has significant reserves of vitamin B12 and loses vitamin B12 slowly. The following case is of a patient who underwent a gastric bypass five years ago and whose hemoglobin (Hgb) dropped from 12.2 g/dL to 4.4 g/dL over six months due to questionable adherence to vitamin supplements. Further work-up showed hemolytic anemia and thrombocytopenia due to a very low vitamin B12 level of 47 pg/mL, with his blood counts improving with vitamin B12 supplementation. The case points to the importance of thinking about vitamin deficiency as a cause of hemolysis to avoid unnecessary procedures. Full article
24 pages, 8502 KiB  
Systematic Review
Comparative Analysis of Endovascular Intervention and Endarterectomy in Patients with Femoral Artery Disease: A Systematic Review and Meta-Analysis
by Nidhruv Ravikumar, Gopika Sreejith, Sharon Hiu Ching Law, Prakhar Anand, Noah Varghese, Samrin Kagdi, Navneet Kang, Mohamed Nashnoush, Sihat Salam and Ibsen Ongidi
Hematol. Rep. 2022, 14(2), 179-202; https://doi.org/10.3390/hematolrep14020026 - 1 Jun 2022
Cited by 3 | Viewed by 3517
Abstract
Peripheral artery disease is a prevalent illness affecting more than 200 million people worldwide. A commonly used technique to manage the condition has been open endarterectomy. However, in recent times, a shift towards minimally invasive techniques has resulted in endovascular intervention as a [...] Read more.
Peripheral artery disease is a prevalent illness affecting more than 200 million people worldwide. A commonly used technique to manage the condition has been open endarterectomy. However, in recent times, a shift towards minimally invasive techniques has resulted in endovascular intervention as a popular alternative. This review aims to assess the safety and efficacy of endovascular intervention when compared with endarterectomy. A systematic review of the articles published in PubMed, Ovid, Embase, and Scopus within the last 10 years was conducted. The PRISMA guidelines were adhered to, and the Newcastle-Ottawa and NICE quality assessment scales were used. A meta-analysis of proportions was performed using the RStudio software (RStudio Team (2021). RStudio: Integrated Development Environment for R, PBC, Boston, MA, USA). Twenty-six studies were included, with a total of 7126 patients (endovascular, 2496; endarterectomy, 4630). Technical success was greater for endarterectomy than endovascular intervention with an odds ratio of 0.38; 95% CI [0.27–0.54]. In terms of safety as well endovascular intervention was better than endarterectomy with an odds ratio of 0.22; 95% CI [0.15 to 0.31] for wound infection. Endovascular intervention is a safe and effective procedure; however, it cannot be considered superior to endarterectomy. Full article
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7 pages, 806 KiB  
Case Report
Unique Presentation of Bortezomib-Associated Thrombotic Microangiopathy Responsive to Therapeutic Plasma Exchange and Eculizumab Therapy
by Robert C. Sterner and William Nicholas Rose
Hematol. Rep. 2022, 14(2), 119-125; https://doi.org/10.3390/hematolrep14020018 - 5 Apr 2022
Cited by 3 | Viewed by 2420
Abstract
Thrombotic microangiopathies (TMA) are a rare group of life-threatening hematological conditions characterized by thrombocytopenia and microangiopathic hemolytic anemia. Although our understanding of the pathophysiology and the availability of diagnostic testing has improved for primary TMAs, such as thrombotic thrombocytopenic purpura, the pathophysiology underlying [...] Read more.
Thrombotic microangiopathies (TMA) are a rare group of life-threatening hematological conditions characterized by thrombocytopenia and microangiopathic hemolytic anemia. Although our understanding of the pathophysiology and the availability of diagnostic testing has improved for primary TMAs, such as thrombotic thrombocytopenic purpura, the pathophysiology underlying secondary TMAs, including drug-induced TMAs (DITMAs), remains less clear. In this case report, we present the unique case of a patient with a history of multiple myeloma that presented four months after the initiation of bortezomib therapy with a bortezomib-associated TMA that responded to therapeutic plasma exchange (TPE) with plasma replacement and eculizumab therapy. This case demonstrates the possible utility of TPE with plasma replacement and eculizumab therapy in DITMA patients that fail to respond following a trial of holding the suspected medication. Full article
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9 pages, 538 KiB  
Case Report
Outcomes of the Pregnancies with Chronic Myeloid Leukemia in the Tyrosine Kinase Inhibitor Era and Literature Review
by Dan Ran Castillo, Daniel Park, Akhil Mehta, Simmer Kaur, Anthony Nguyen and Mojtaba Akhtari
Hematol. Rep. 2022, 14(1), 45-53; https://doi.org/10.3390/hematolrep14010008 - 20 Mar 2022
Cited by 3 | Viewed by 6090
Abstract
Chronic myeloid leukemia (CML) is a clonal myeloproliferative neoplasm (MPN) that accounts for 10% of pregnancy-associated leukemias. The Philadelphia chromosome balanced translocation, t (9:22) (q34; q11.2), is the classic mutation seen in CML. The BCR-ABL oncoprotein encoded by this mutation is a constitutively [...] Read more.
Chronic myeloid leukemia (CML) is a clonal myeloproliferative neoplasm (MPN) that accounts for 10% of pregnancy-associated leukemias. The Philadelphia chromosome balanced translocation, t (9:22) (q34; q11.2), is the classic mutation seen in CML. The BCR-ABL oncoprotein encoded by this mutation is a constitutively active tyrosine kinase. Tyrosine kinase inhibitor (TKI) therapy is considered a first-line treatment for CML. However, the literature has revealed risks of teratogenicity with TKI therapy during pregnancy. Understanding the risks and benefits of TKI therapy and alternative therapies such as interferon-alpha (IFN-α) will help clinicians and pregnant patients develop a personalized CML treatment plan. This manuscript presents a case series detailing the management of five pregnancies in two pregnant patients with CML and a literature review of CML management in pregnancy. Full article
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7 pages, 1207 KiB  
Case Report
Challenging Diagnosis of Pure Erythroid Leukemia: A Case Report and Literature Review
by Shingo Sato, Masayuki Kobayashi, Ken Suzaki, Ittoku Nanke and Nobuharu Kosugi
Hematol. Rep. 2022, 14(1), 38-44; https://doi.org/10.3390/hematolrep14010007 - 19 Mar 2022
Cited by 2 | Viewed by 3053
Abstract
Pure erythroid leukemia (PEL) is an extremely rare type of acute myeloid leukemia (AML), accounting for fewer than 1% of all AML cases. A 72-year-old man presented with severe fatigue. His bone marrow aspiration contained myeloperoxidase negative abnormal cells that were aggregating and [...] Read more.
Pure erythroid leukemia (PEL) is an extremely rare type of acute myeloid leukemia (AML), accounting for fewer than 1% of all AML cases. A 72-year-old man presented with severe fatigue. His bone marrow aspiration contained myeloperoxidase negative abnormal cells that were aggregating and depicting epithelial adhesion, suggesting the possibility of solid tumor metastasis. His general condition deteriorated during medical diagnosis, and he died soon after starting chemotherapy. PEL appeared to be the definitive diagnosis after evaluating the histopathological findings, which were obtained after his death. With atypical morphological features, immunophenotypic and karyotypic approaches must be integrated for PEL assessment. Full article
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7 pages, 392 KiB  
Article
Blood Group Type Association with Head and Neck Cancer
by Gaube Alexandra, Michire Alexandru, Calangiu Filip Stefan, Draghia Petruta-Maria, Burlacu Mihnea Gabriel, Georgescu Dragos-Eugen and Georgescu Mihai Teodor
Hematol. Rep. 2022, 14(1), 24-30; https://doi.org/10.3390/hematolrep14010005 - 2 Mar 2022
Cited by 14 | Viewed by 3789
Abstract
Background: We conducted an analysis to check whether the ABO blood group impacts the susceptibility or protection against different types of head and neck cancers. Method: We analyzed the medical records of 61,899 cancer patients from “Prof. Dr. Alexandru Trestioreanu” Institute of Oncology [...] Read more.
Background: We conducted an analysis to check whether the ABO blood group impacts the susceptibility or protection against different types of head and neck cancers. Method: We analyzed the medical records of 61,899 cancer patients from “Prof. Dr. Alexandru Trestioreanu” Institute of Oncology from Bucharest, along with the corresponding blood group type. Data were scraped using Python. For analysis, we used Chi-square test. Results: The blood group count was A (245, 45.12%) followed by 0 (160, 24.66%), B (110, 20.26%), and AB (28, 5.16%). Hypopharyngeal cancer was associated with B group, oral cavity cancer was associated with a lower risk in patients with B group while AB patients had a higher risk for oral cavity cancer (χ2 = 36.136, df = 18, p = 0.007). Conclusion: Blood group B is associated with an increased incidence for hypopharyngeal cancer, whereas, for the oral cavity, was associated lower incidence. Blood antigen A is associated with a higher risk of oral cavity cancer development, independent of B blood antigen. Full article
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