Hematology Reports

12 pages, 1595 KiB

Open AccessEditor’s ChoiceArticle

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 4142

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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8 pages, 2215 KiB

Open AccessEditor’s ChoiceCase Report

Extramedullary T-lymphoblastic Crisis in a Myelodysplastic/Myeloproliferative Neoplasm with a t(12;22)/MN1::ETV6 Translocation

by Ana Carolina Freitas, Tiago Maia, Joana Desterro, Francesca Pierdomenico, Albertina Nunes, Isabelina Ferreira, José Cabeçadas and Maria Gomes da Silva

Hematol. Rep. 2023, 15(1), 212-219; https://doi.org/10.3390/hematolrep15010022 - 14 Mar 2023

Cited by 2 | Viewed by 2183

Abstract

Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are not a single disease, but rather a heterogenous group of entities which are increasingly subclassified according to recurrent genetic abnormalities. Chromosomal translocations involving meningioma 1 (MN1) and ETS variant 6 (ETV6) genes are extremely rare, [...] Read more.

Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are not a single disease, but rather a heterogenous group of entities which are increasingly subclassified according to recurrent genetic abnormalities. Chromosomal translocations involving meningioma 1 (MN1) and ETS variant 6 (ETV6) genes are extremely rare, but recurrent in myeloid neoplasms. We describe the case of a patient with a myelodysplastic/myeloproliferative neoplasm with neutrophilia, who developed an extramedullary T-lymphoblastic crisis with the t(12;22)(p13;q12) translocation as the only cytogenetic abnormality. This case shares several clinical and molecular features with myeloid/lymphoid neoplasms with eosinophilia. The treatment of this patient was challenging, as the disease proved to be highly refractory to chemotherapy, with allogenic stem cell transplantation as the only curative option. This clinical presentation has not been reported in association with these genetic alterations and supports the concept of a hematopoietic neoplasm originating in an early uncommitted precursor cell. Additionally, it stresses the importance of molecular characterization in the classification and prognostic stratification of these entities. Full article

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13 pages, 4342 KiB

Open AccessEditor’s ChoiceCase 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 3737

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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8 pages, 733 KiB

Open AccessEditor’s ChoiceCase Report

Hypoxia-Inducible Factor-Prolyl-Hydroxylase and Sodium-Glucose Cotransporter 2 Inhibitors for Low-Risk Myelodysplastic Syndrome-Related Anemia in Patients with Chronic Kidney Disease: A Report of Three Cases

by Satoshi Yamasaki and Takahiko Horiuchi

Hematol. Rep. 2023, 15(1), 180-187; https://doi.org/10.3390/hematolrep15010019 - 6 Mar 2023

Cited by 2 | Viewed by 2479

Abstract

Although daprodustat, a hypoxia-inducible factor prolyl hydroxylase inhibitor, and dapagliflozin, a sodium-glucose cotransporter 2 inhibitor, have been approved for the treatment of renal anemia in Japan, their efficacy and safety for patients aged 80 years or older with low-risk myelodysplastic syndrome (MDS)-related anemia [...] Read more.

Although daprodustat, a hypoxia-inducible factor prolyl hydroxylase inhibitor, and dapagliflozin, a sodium-glucose cotransporter 2 inhibitor, have been approved for the treatment of renal anemia in Japan, their efficacy and safety for patients aged 80 years or older with low-risk myelodysplastic syndrome (MDS)-related anemia have not been demonstrated. Our case series comprised two men and one woman aged >80 years with low-risk MDS-related anemia and diabetic mellitus (DM)-related chronic kidney disease who were dependent on red blood cell transfusions and in whom erythropoiesis-stimulating agents had been insufficient. All three patients received daprodustat and additional dapagliflozin achieved red blood cell transfusion independence and were followed up for >6 months. Daily oral daprodustat was well tolerated. There were no fatalities or progression to acute myeloid leukemia during the >6-month follow-up after daprodustat initiation. On the basis of these outcomes, we consider 24 mg of daprodustat combined with 10 mg of dapagliflozin daily an effective form of treatment for low-risk MDS-related anemia. Further studies are required to clarify the synergistic effects of daprodustat and dapagliflozin, which correct chronic kidney disease-related anemia by promoting endogenous erythropoietin production and normalizing iron metabolism to manage low-risk MDS in the long term. Full article

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8 pages, 411 KiB

Open AccessEditor’s ChoiceCase Report

A Case Report of Ropeginterferon Alfa-2b for Polycythemia Vera during Pregnancy

by Su-Yeon Bang and Sung-Eun Lee

Hematol. Rep. 2023, 15(1), 172-179; https://doi.org/10.3390/hematolrep15010018 - 2 Mar 2023

Cited by 2 | Viewed by 3894

Abstract

Myeloproliferative neoplasms (MPN) such as essential thrombocythemia (ET) and polycythemia vera (PV) are rare during pregnancy. However, they are harmful because they are associated with an increased risk of thromboembolic, hemorrhagic, or microcirculatory disturbances or placental dysfunction leading to fetal growth restriction or [...] Read more.

Myeloproliferative neoplasms (MPN) such as essential thrombocythemia (ET) and polycythemia vera (PV) are rare during pregnancy. However, they are harmful because they are associated with an increased risk of thromboembolic, hemorrhagic, or microcirculatory disturbances or placental dysfunction leading to fetal growth restriction or loss. Low-dose aspirin and low-molecular-weight heparin (LMWH) are recommended to reduce pregnancy complications, and interferon (IFN) is the only treatment option for cytoreductive therapy based on the likelihood of live birth in pregnant women with MPN. Since ropeginterferon alfa-2b is the only available IFN in South Korea, we present a case report of ropeginterferon alfa-2b use during pregnancy in an MPN patient. A 40-year-old woman who had been diagnosed with low-risk PV in 2017 and had been maintained on phlebotomy, hydroxyurea (HU), and anagrelide (ANA) for 4 years was confirmed as 5 weeks pregnant on 9 December 2021. After stopping treatment with HU and ANA, the patient showed a rapid increase in platelet count (1113 × 10⁹/L to 2074 × 10⁹/L, normal range, 150–450 × 10⁹/L) and white blood cell count (21.93 × 10⁹/L to 35.55 × 10⁹/L, normal range, 4.0–10.0 × 10⁹/L). Considering the high risk of complications, aggressive cytoreductive treatment was required, for which we chose ropeginterferon alfa-2b, as it is the only available IFN agent in South Korea. The patient underwent 8 cycles of ropeginterferon alfa-2b over 6 months during pregnancy and delivered without any neonatal or maternal complications. This case report highlights the importance of considering treatment options for MPN patients who are pregnant or planning a pregnancy, as well as the need for further investigation into the safety and efficacy of ropeginterferon alfa-2b in this population. Full article

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9 pages, 264 KiB

Open AccessEditor’s ChoiceArticle

Neoplastic and Autoimmune Comorbidities in Patients with Primary Cutaneous B-Cell Lymphoma

by Roberto Mazzetto, Jacopo Tartaglia, Alvise Sernicola and Mauro Alaibac

Hematol. Rep. 2023, 15(1), 157-165; https://doi.org/10.3390/hematolrep15010016 - 27 Feb 2023

Cited by 1 | Viewed by 2066

Abstract

Primary cutaneous B-cell lymphomas (PCBCLs) constitute a rare subset of non-Hodgkin lymphoma (NHL), with distinctive clinical and biological characteristics. The risk of autoimmune or neoplastic comorbidities in subjects with NHL has been extensively reported in the literature, but the data available are not [...] Read more.

Primary cutaneous B-cell lymphomas (PCBCLs) constitute a rare subset of non-Hodgkin lymphoma (NHL), with distinctive clinical and biological characteristics. The risk of autoimmune or neoplastic comorbidities in subjects with NHL has been extensively reported in the literature, but the data available are not directly applicable to PCBCLs. The aim of our study was to determine the frequency of relevant medical conditions, with a primary focus on autoimmune and neoplastic disorders, in subjects with PCBCL. We performed a retrospective observational study involving 56 patients diagnosed histologically with PCBCL and 54 sex- and age-matched controls. Our results show a statistically significant association for neoplastic comorbidities in general (41.1% vs. 22.2%, p = 0.034) and hematological malignancies specifically (19.6% vs. 1.9%, p = 0.0041) with PCBCL compared to controls. We did not highlight a statistically significant difference in the frequency of autoimmune comorbidities (21.4% vs. 9.3%, p = 0.1128) and of chronic viral hepatitis (7.1% vs. 0, p = 0.1184). Finally, type 2 diabetes (19.6% vs. 1.9%, p = 0.0041) was significantly associated with PCBCL. Our preliminary data supporting the association between PCBCLs and neoplastic disorders suggest that altered immune surveillance may be a common predisposing mechanism. Full article

6 pages, 512 KiB

Open AccessEditor’s ChoiceBrief 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 3428

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

Open AccessEditor’s ChoiceArticle

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 3191

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

Open AccessEditor’s ChoiceArticle

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 2403

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

Open AccessEditor’s ChoiceReview

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 4536

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

Open AccessEditor’s ChoiceReview

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 4506

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

Open AccessEditor’s ChoiceArticle

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 2229

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

Open AccessEditor’s ChoiceCase 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 7232

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

Open AccessEditor’s ChoiceArticle

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 2854

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

Open AccessEditor’s ChoiceCase 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 3144

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

Open AccessEditor’s ChoiceReview

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 3501

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

Open AccessEditor’s ChoiceCase 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 2439

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

Open AccessEditor’s ChoiceCase 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 3963

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

Open AccessEditor’s ChoiceCase 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 3578

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

Open AccessEditor’s ChoiceSystematic 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 3556

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

Open AccessEditor’s ChoiceCase 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 2447

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

Open AccessEditor’s ChoiceCase 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 6162

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

Open AccessEditor’s ChoiceCase 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 3092

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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Graphical abstract

7 pages, 392 KiB

Open AccessEditor’s ChoiceArticle

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 3824

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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