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Hematology Reports
Volume 17
Issue 6
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Hematol. Rep., Volume 17, Issue 6 (December 2025) – 17 articles

Cover Story (view full-size image): There are two different types of myelodysplastic neoplasms (MDSs) associated with the deletion of the chromosome 5q region—isolated 5 q deletion (MDS-iso5q) and 5q deletion—observed in association with complex chromosomal abnormalities (MDS-CK). MDS-iso5q usually originates from 5q del as a primary pathogenic event, while MDS-CK usually originates from TP53 mutations as a primary pathogenic event, followed by del5q and other chromosomal abnormalities in the context of generalized chromosomal instability. MDS-iso5q has a better prognosis than MDS-CK. Both MDS-iso5q and MDS-CK are heterogenous at the molecular level, and the characterization of their molecular heterogeneity is fundamental to defining an optimal therapeutic strategy. View this paper
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11 pages, 474 KB  
Review
The Use of Single Dose of Rasburicase for the Prophylaxis and Treatment of Tumor Lysis Syndrome in Pediatric Patients: A Narrative Review
by Anselm Chi-wai Lee
Hematol. Rep. 2025, 17(6), 71; https://doi.org/10.3390/hematolrep17060071 - 18 Dec 2025
Viewed by 2509
Abstract
Background/Objectives: Rasburicase is licensed for the management of tumor lysis syndrome (TLS) at a daily dose of 0.2 mg/kg intravenously for five days. The use of a single-dose treatment is popular in adult oncology but information in pediatric use is limited. Methods [...] Read more.
Background/Objectives: Rasburicase is licensed for the management of tumor lysis syndrome (TLS) at a daily dose of 0.2 mg/kg intravenously for five days. The use of a single-dose treatment is popular in adult oncology but information in pediatric use is limited. Methods: From a literature search, all case reports and series, and comparative studies in which pediatric oncology patients received a single dose of rasburicase were selected for further analysis. Treatment success was determined by normalization of serum uric acid in the absence of serious complications. Results: Twelve articles with a total of 243 children were included. A fixed-dose regimen was used in 195, while 153 received weight-based dosing. With fixed dosing, successful treatment was seen in 91.8% and 82.9% at rasburicase doses ≥3 mg and 1.5 mg, respectively (p = 0.23). However, there were four mortalities in the lower-dose group. For weight-based dosing, success was observed in 89.2% and 66.7% at doses ≥0.15 mg/kg and <0.15 mg/kg, respectively (p = 0.0029). One child required dialysis in the lower-dose group. Conclusions: Single dose of rasburicase for the prophylaxis and treatment of TLS in pediatric oncology is an appealing approach with potentially less financial impact and drug toxicity. A fixed dose of at least 3 mg or 0.15 mg/kg by body weight is recommended. Full article
10 pages, 416 KB  
Brief Report
Characteristics and Outcomes of Acute Leukemias in Adolescents and Young Adults with Down Syndrome: A Single-Center Experience
by Marie Nour Karam, Sandra K. Althouse, Madeline G. Andrews, Jenny Chen and Sandeep Batra
Hematol. Rep. 2025, 17(6), 70; https://doi.org/10.3390/hematolrep17060070 - 18 Dec 2025
Viewed by 676
Abstract
Background/Objectives: Acute leukemias in adolescents and young adults (AYAs) with Down Syndrome (DS) are understudied. Methods: This was a single-center, retrospective cohort study. Medical records for pediatric DS (n = 41) and AYA-DS (n = 7) treated with a pediatric [...] Read more.
Background/Objectives: Acute leukemias in adolescents and young adults (AYAs) with Down Syndrome (DS) are understudied. Methods: This was a single-center, retrospective cohort study. Medical records for pediatric DS (n = 41) and AYA-DS (n = 7) treated with a pediatric chemotherapy regimen for acute leukemia were evaluated. Results: Two-year event-free survival (EFS) in AYA DS acute leukemia patients was lower than that in their pediatric DS counterparts (28.6% (Confidence Interval (CI) 4.1, 61.2) vs. 84.9% (CI 69.5, 92.9); p = 0.002). Conclusions: Additional research is needed to improve outcomes in AYA DS leukemia. Full article
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9 pages, 337 KB  
Case Report
Ibrutinib-Associated Liver Injury in a Patient with Chronic Lymphocytic Leukemia: Clinical Course and Therapeutic Approach
by Antonio Frolli, Guido Parvis, Martina Bullo, Selene Grano, Giovanni Fornari, Valentina Bonuomo, Daniela Cilloni and Carmen Fava
Hematol. Rep. 2025, 17(6), 69; https://doi.org/10.3390/hematolrep17060069 - 11 Dec 2025
Cited by 1 | Viewed by 1745
Abstract
Background: Ibrutinib, a Bruton’s tyrosine kinase inhibitor (BTKi), has revolutionized the treatment of Chronic Lymphocytic Leukemia (CLL), yet hepatotoxicity remains a rare and poorly characterized adverse event. Case Presentation: We report the case of a 54-year-old man with progressive CLL and radiologically confirmed [...] Read more.
Background: Ibrutinib, a Bruton’s tyrosine kinase inhibitor (BTKi), has revolutionized the treatment of Chronic Lymphocytic Leukemia (CLL), yet hepatotoxicity remains a rare and poorly characterized adverse event. Case Presentation: We report the case of a 54-year-old man with progressive CLL and radiologically confirmed hepatic involvement who developed acute hepatocellular injury during ibrutinib monotherapy. Baseline liver tests showed mild abnormalities attributed to hepatic steatosis and leukemic infiltration. After approximately 10–12 weeks of ibrutinib (420 mg/day), transaminases markedly increased (ALT 660 U/L, AST 326 U/L), while bilirubin and synthetic function remained normal. Viral, autoimmune, and obstructive causes were excluded. Stepwise dose reductions to 140 mg/day provided limited benefit. The addition of prednisone (50 mg/day) led to rapid biochemical improvement. Ibrutinib was successfully re-escalated to 280 mg/day alongside venetoclax initiation, maintaining disease control without recurrence of liver injury. Discussion: The temporal relationship, exclusion of alternative causes, and corticosteroid responsiveness suggest an ibrutinib-induced liver injury, possibly exacerbated by pre-existing hepatic steatosis and leukemic infiltration. Conclusions: This case underscores the multifactorial pathogenesis of BTKi-related hepatotoxicity and highlights the potential role of corticosteroids in management. Prompt recognition, stepwise dose adjustment, and corticosteroid therapy may enable continued treatment and sustained disease control in selected patients. Full article
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8 pages, 762 KB  
Case Report
Double Trouble: The First Reported Case of Evans Syndrome Following RSV Vaccination
by Mohammad Abu-Tineh, Deepika Beereddy, Ilse Ivonne Saldivar Ruiz and Divya Samat
Hematol. Rep. 2025, 17(6), 68; https://doi.org/10.3390/hematolrep17060068 - 1 Dec 2025
Cited by 1 | Viewed by 1874
Abstract
Background: Evans syndrome is a rare autoimmune disease characterized by immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA), and autoimmune neutropenia, typically triggered by an episode of immune dysregulation or multiple other factors. We present what appears to be the first reported case of [...] Read more.
Background: Evans syndrome is a rare autoimmune disease characterized by immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA), and autoimmune neutropenia, typically triggered by an episode of immune dysregulation or multiple other factors. We present what appears to be the first reported case of Evans syndrome developing in a 66-year-old female following respiratory syncytial virus (RSV) vaccination. Case Presentation: A 66-year-old female presented with a petechial rash on her arms, legs, and face. Laboratory tests revealed a platelet count of 1 × 109/L, significantly lower than her historical baseline of >200 × 109/L. On hospital day 4, her hemoglobin declined from 14.3 g/dL to 9.9 g/dL, with laboratory evidence of hemolysis, including elevated bilirubin, low haptoglobin, and increased lactate dehydrogenase (LDH). Bone marrow biopsy revealed megakaryocytic hyperplasia consistent with ITP, along with a small polyclonal B-cell population lacking CD20 expression. Imaging was unremarkable, showing no interval changes aside from stable pre-existing pulmonary nodules and no lymphadenopathy. These findings supported a diagnosis of Evans syndrome. Initial therapy with dexamethasone and intravenous immunoglobulin (IVIG) for presumed ITP was ineffective. Due to refractory thrombocytopenia, the patient initially received one dose of rituximab, followed by one dose of romiplostim. Subsequently, the patient received rituximab infusions every week at a rate of 375 mg/m2 for four doses, as well as prednisone at a dose of 1 mg/kg/day. Within five weeks, her blood count returned to normal. Conclusions: This case raises concern for a potential temporal association between RSV vaccination and the onset of Evans syndrome. It underscores the need for heightened clinical awareness and further investigation into immune-mediated hematologic complications following RSV immunization. Full article
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28 pages, 807 KB  
Review
Clinical and Biological Insights into Myelodysplastic Neoplasms Associated with Deletions of Chromosome 5q Region
by Ugo Testa, Germana Castelli and Elvira Pelosi
Hematol. Rep. 2025, 17(6), 67; https://doi.org/10.3390/hematolrep17060067 - 29 Nov 2025
Cited by 1 | Viewed by 3251
Abstract
The only cytogenetic alteration defining a subtype of a myelodysplastic syndrome is represented by the deletion of the long arm of chromosome 5 (del(5q)), now classified as MDS with isolated del(5q). This subtype is associated with a peculiar phenotype mainly dependent on the [...] Read more.
The only cytogenetic alteration defining a subtype of a myelodysplastic syndrome is represented by the deletion of the long arm of chromosome 5 (del(5q)), now classified as MDS with isolated del(5q). This subtype is associated with a peculiar phenotype mainly dependent on the haploinsufficiency of several genes located on the deleted arm of chromosome 5. These patients show a good prognosis and respond to treatment with lenalidomide, but some cases progress to acute myeloid leukemia. Molecular studies have, in part, elucidated the heterogeneity of MDS with isolated del(5q), mainly related to the association with different co-mutations that may affect leukemic transformation and survival. In other MDS patients, del(5q) is combined with other chromosomal abnormalities, giving rise to a condition of complex karyotype, associated with frequent TP53 mutations and with a poor prognosis. Two different molecular pathways seem to be responsible for the generation of MDS with isolated del(5q) or of MDS with del(5q) associated with a complex karyotype. Full article
(This article belongs to the Special Issue Innovations in Hematologic Oncology: SOHO Italy Perspectives)
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11 pages, 812 KB  
Review
Acute Promyelocytic Leukemia: Pathophysiology, Diagnosis and Clinical Management
by Meryeme Abddaoui, Youssef Aghlallou, Imane Tlemçani and Moncef Amrani Hassani
Hematol. Rep. 2025, 17(6), 66; https://doi.org/10.3390/hematolrep17060066 - 28 Nov 2025
Cited by 4 | Viewed by 12304
Abstract
Background/Objectives: Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia characterized by the t(15;17)(q24;q21) translocation, generating the PML::RARA fusion gene that blocks myeloid differentiation and drives leukemogenesis. Despite advances in therapy, early mortality remains a major challenge due to [...] Read more.
Background/Objectives: Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia characterized by the t(15;17)(q24;q21) translocation, generating the PML::RARA fusion gene that blocks myeloid differentiation and drives leukemogenesis. Despite advances in therapy, early mortality remains a major challenge due to severe coagulopathy. This review aims to summarize recent insights into APL pathophysiology, diagnostic approaches, and management strategies. Methods: We performed a comprehensive review of the literature addressing the molecular mechanisms of APL, its associated coagulopathy, and current diagnostic and therapeutic standards, with a focus on evidence-based recommendations for clinical practice. Results: The hallmark PML: RARA oncoprotein disrupts nuclear body function and retinoic acid signaling, resulting in differentiation arrest and apoptosis resistance. APL-associated coagulopathy arises from overexpression of tissue factor, release of cancer procoagulant, inflammatory cytokines, and annexin II-mediated hyperfibrinolysis. Diagnosis requires integration of cytomorphology, immunophenotyping, coagulation studies, and molecular confirmation. Immediate initiation of all-trans-retinoic acid (ATRA) upon clinical suspicion, combined with aggressive supportive care, is critical to control bleeding risk. Conclusions: APL is now a highly curable leukemia when recognized early and treated with targeted therapy. Rapid diagnosis, prompt ATRA administration, and meticulous hemostatic support are essential to reduce early mortality. Further refinements in minimal residual disease monitoring are expected to improve patient outcomes. Full article
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15 pages, 3410 KB  
Case Report
Hodgkin Lymphoma Associated with Common Variable Immunodeficiency: The Role of Early Diagnosis and Multidisciplinary Management
by Dávid Tóthfalusi, Anita Gulyás, Anna Koncz, Éva Zöld, Árpád Illés and Zsófia Miltényi
Hematol. Rep. 2025, 17(6), 65; https://doi.org/10.3390/hematolrep17060065 - 27 Nov 2025
Viewed by 2538
Abstract
Background/Objectives: Hodgkin lymphoma (HL) represents a rare but clinically significant complication in patients with Common Variable Immunodeficiency (CVID). Immune dysregulation, impaired viral control, and Epstein–Barr virus (EBV) infection may contribute to pathogenesis and adversely affect treatment tolerance. This case-based review aims to [...] Read more.
Background/Objectives: Hodgkin lymphoma (HL) represents a rare but clinically significant complication in patients with Common Variable Immunodeficiency (CVID). Immune dysregulation, impaired viral control, and Epstein–Barr virus (EBV) infection may contribute to pathogenesis and adversely affect treatment tolerance. This case-based review aims to highlight the impact of early CVID recognition and multidisciplinary management on outcomes in CVID-associated HL. Methods: A retrospective screening of 224 patients with HL treated at our institution between 2010 and 2023 identified two individuals with CVID and EBV-positive HL. These cases are presented in detail and contextualized within a structured review of the published literature. Results: The first patient, diagnosed with CVID prior to HL onset, received immunoglobulin replacement and a modified chemotherapy regimen substituting bleomycin with brentuximab vedotin, resulting in sustained complete remission. The second patient, in whom CVID was recognized only after HL relapse, experienced recurrent infections, intolerance to therapy, and fatal disease progression despite treatment with brentuximab vedotin, checkpoint inhibition, and rituximab. The literature review revealed only eight comparable cases, underscoring the rarity and complexity of this association. Conclusions: Early identification of CVID facilitates infection control and enhances tolerance to HL therapy, thereby improving clinical outcomes. Multidisciplinary, individualized management and incorporation of targeted agents are pivotal in optimizing care for this vulnerable population. Full article
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8 pages, 1671 KB  
Case Report
Diagnostic Challenges in a Young Man with a Suspected Mast Cell Disorder, Dysplastic Bone Marrow Morphology, and a ZRSR2 Mutation
by Riccardo Dondolin, Nawar Maher, Annalisa Andorno, Sayed Masoud Sayedi, Mohammad Reshad Nawabi, Andrea Patriarca, Gianluca Gaidano and Riccardo Moia
Hematol. Rep. 2025, 17(6), 64; https://doi.org/10.3390/hematolrep17060064 - 25 Nov 2025
Viewed by 969
Abstract
Background and Clinical Significance: Mastocytosis and mast cell activation syndrome (MCAS) include conditions in which patients manifest signs, symptoms, and laboratory findings consistent with mast cell activation and can only be diagnosed in the presence of specific criteria. Mutations of ZRSR2, a [...] Read more.
Background and Clinical Significance: Mastocytosis and mast cell activation syndrome (MCAS) include conditions in which patients manifest signs, symptoms, and laboratory findings consistent with mast cell activation and can only be diagnosed in the presence of specific criteria. Mutations of ZRSR2, a gene involved in RNA splicing, are not closely associated with mast cell disorders, but rather with myelodysplastic syndromes development. Case Presentation: We report a case of a 37-year-old man who was referred to our institution for anaphylaxis after a bee sting and elevated serum tryptase levels (17.8 ng/mL in the first sample and 19.2 ng/mL in the second sample). Complete blood count was unremarkable. Bone marrow biopsy showed signs of dysplasia and some CD25+ mast cells. ASO-qPCR and targeted myeloid NGS analysis did not detect the KIT p.D816V mutation, but rather showed the presence of a pathogenetic variant of the ZRSR2 gene (p.S447_R448del) with a variant allele frequency of 7.4%. Mastocytosis could not be diagnosed based on the established diagnostic criteria. The patient’s symptoms were not recurrent and tryptase release was not event-related; therefore, a diagnosis of MCAS could not be made either. Taken together, these findings led to the diagnosis of clonal hematopoiesis of indeterminate potential (CHIP). A watch and wait strategy consisting of clinical evaluations, blood tests, and cardiovascular risk assessment was initiated. Conclusions: This case report highlights the importance of combining clinical and laboratory findings, hematopathology, and molecular analyses to establish the most probable diagnosis in challenging cases. It also underscores the possible relevance of identifying predisposing conditions, such as CHIP, in order to guide counseling and follow-up strategy. Full article
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12 pages, 815 KB  
Article
Mucosal-Associated Lymphoid Tissue Lymphoma in Southeast Asia: A 15-Year Retrospective Multicenter Study
by Kannadit Prayongratana, Tanapun Thamgrang, Chonlada Laoruangroj, Lalita Norasetthada, Thanawat Rattanathammethee, Udomsak Bunworasate, Kitsada Wudhikarn, Jakrawadee Julamanee, Panarat Noiperm, Suporn Chuncharunee, Pimjai Niparuck, Archrob Khuhapinant, Noppadol Siritanaratkul, Piyapong Kanya, Kanchana Chansung, Chittima Sirijerachai, Dusit Jit-Uaekul, Juthatip Chaloemwong, Nonglak Kanitsap, Peerapon Wong, Nisa Makruasi, Somchai Wongkhantee, Tawatchai Suwanban and Tanin Intragumtornchaiadd Show full author list remove Hide full author list
Hematol. Rep. 2025, 17(6), 63; https://doi.org/10.3390/hematolrep17060063 - 25 Nov 2025
Cited by 1 | Viewed by 1889
Abstract
Objective: To describe the epidemiology, survival rate, and prognostic factors of mucosal-associated lymphoid tissue (MALT) lymphoma. Patients and Methods: This investigation utilized the Thai Lymphoma Study Group (TLSG) registry to gather data on patients diagnosed with MALT lymphoma. The analysis included demographic details, [...] Read more.
Objective: To describe the epidemiology, survival rate, and prognostic factors of mucosal-associated lymphoid tissue (MALT) lymphoma. Patients and Methods: This investigation utilized the Thai Lymphoma Study Group (TLSG) registry to gather data on patients diagnosed with MALT lymphoma. The analysis included demographic details, therapeutic interventions, and survival statistics. Results: The TLSG registry prospectively included 8404 patients with lymphoma. Among them, marginal-zone lymphoma (MZL) was the second most common subtype, with 670 histologically confirmed cases, accounting for 8.0% of the total cohort. An analysis of the MZL subtypes showed that MALT lymphoma was the most common, accounting for 77.8% of the diagnoses. This was followed by nodal MZL at 17.5% and splenic MZL at 7.7%. The distribution of primary disease sites indicated that the ocular adnexa (49.2%), stomach (12.9%), and sinonasal region (12.5%) were the three most common locations. Three variables were found to be statistically significant predictors of survival in the multivariate analysis: ECOG performance status > 2, age exceeding 65 years, and involvement of more than two extranodal organs. These identified prognostic factors were further assessed for their effect on overall survival (OS) and progression-free survival (PFS). A risk classification was established: the low-risk group comprised patients with zero identified risk factors, whereas the high-risk group included patients who had any of the specified risk factors. A comparison of five-year survival rates showed significantly more favorable outcomes for low-risk patients who had a PFS of 83.3% (vs. 66.1%, p = 0.028) and an OS of 97.8% (vs. 76.7%, p < 0.001) compared to the high-risk group. Conclusions: In this cohort, where MZL was the second most common lymphoma and MALT lymphoma was the predominant subtype, our analysis revealed that patients with no risk factors experienced statistically significant improvements in both PFS and OS. Full article
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9 pages, 738 KB  
Article
Classic Coagulation Traits Vary According to Rh(D) (But Not ABO) Blood Groups
by Gilberto Santos Morais-Junior, Patrícia Dias da Silva, Mayara Barbosa da Silva, Jamila Reis de Oliveira, Andersen Charles Daros, Ciro Martins Gomes and Otávio Toledo Nóbrega
Hematol. Rep. 2025, 17(6), 62; https://doi.org/10.3390/hematolrep17060062 - 15 Nov 2025
Viewed by 1343
Abstract
Background: This study evaluated possible variations in classic blood coagulation parameters according to groups formed from the main erythrocyte antigen systems. Methods: Consecutive patients admitted to a transfusion hemotherapy service at a private hospital in the Brazilian Federal District were evaluated for coagulation [...] Read more.
Background: This study evaluated possible variations in classic blood coagulation parameters according to groups formed from the main erythrocyte antigen systems. Methods: Consecutive patients admitted to a transfusion hemotherapy service at a private hospital in the Brazilian Federal District were evaluated for coagulation profile and blood type according to routine laboratory practices. The international normalized ratio (INR), the activated partial thromboplastin time (APTT) and the prothrombin time (PT) were compared according to the ABO blood group and the Rh factor in analyses controlled for classic influencers such as age, sex and comorbidities. Results: No significant differences in coagulation were found between groups defined by the ABO antigen system, despite a body of evidence in favor of this correlation. Rh-positive individuals showed increased mean values in PT (13.7 vs. 12.6 s), in APTT (32.0 vs. 30.1 s) and in INR (1.23 vs. 1.15 s) when compared to the Rh-negative counterparts. Conclusions: Our results suggest a lowered rate of coagulation among Rh-positive individuals, possibly owing to inhibitory effects of the Rh(D) erythrocyte antigen on the coagulation pathway. Full article
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22 pages, 21230 KB  
Article
PD-1 Expression Promotes Immune Evasion in B-ALL
by Ana Casado-García, Gonzalo García-Aguilera, Julio Pozo, Ninad Oak, Susana Barrena, Belén Ruiz-Corzo, Jaanam Lalchandani, Ana Chamorro-Vera, Ana Castillo-Robleda, Beatriz Soriano, Silvia Alemán-Arteaga, Elena G. Sánchez, Jorge Martínez-Cano, Andrea López-Álvarez de Neyra, Paula Somoza-Cotillas, Oscar Blanco, Susana Riesco, Pablo Prieto-Matos, Francisco Javier García Criado, María Begoña García Cenador, César Cobaleda, Carolina Vicente-Dueñas, Kim E Nichols, Alberto Orfao, Manuel Ramírez-Orellana and Isidro Sánchez-Garcíaadd Show full author list remove Hide full author list
Hematol. Rep. 2025, 17(6), 61; https://doi.org/10.3390/hematolrep17060061 - 12 Nov 2025
Viewed by 2326
Abstract
Background/Objectives: In children developing B-cell acute lymphoblastic leukemia (B-ALL), an immune evasion event takes place where otherwise “silent” preleukemic cells undergo a malignant transformation while escaping immune control, often through unknown mechanisms. Methods and Results: Here, we identify the upregulation of PD-1 expression [...] Read more.
Background/Objectives: In children developing B-cell acute lymphoblastic leukemia (B-ALL), an immune evasion event takes place where otherwise “silent” preleukemic cells undergo a malignant transformation while escaping immune control, often through unknown mechanisms. Methods and Results: Here, we identify the upregulation of PD-1 expression in preleukemic cells, triggered by Pax5 inactivation in mice and correlating with the time of conversion to leukemia, as a novel marker that favors leukemia evasion. This increase in PD-1 expression is apparent across diverse molecular B-ALL subtypes, both in mice and humans. PD-1 is not required for B-cell leukemogenesis, but, in the absence of PD-1, tumor cells express NK cell inhibitory receptors, highlighting the necessity for leukemic cells to evade the host’s NK immune response in order to exit the bone marrow. PD-1 expression reduces natural antitumor immune responses, but it sensitizes leukemic cells to immune checkpoint blockade strategies in mice and humans. PD-1 targeting confers clinical benefits by restoring NK-mediated tumor cell killing in vitro and eliminating tumor cells in vivo in mice engrafted with B-ALL. Conclusions: These results identify PD-1 as a new therapeutic target against leukemic progression, providing new opportunities for the treatment and possibly also the prevention of childhood B-ALL. Full article
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12 pages, 1263 KB  
Article
AI Improves Agreement and Reduces Time for Quantifying Metabolic Tumour Burden in Hodgkin Lymphoma
by May Sadik, Sally F. Barrington, Johannes Ulén, Olof Enqvist, Elin Trägårdh, Babak Saboury, Anne Lerberg Nielsen, Annika Loft, Jose Luis Loaiza Gongora, Jesus Lopez Urdaneta, Rajender Kumar, Martijn van Essen and Lars Edenbrandt
Hematol. Rep. 2025, 17(6), 60; https://doi.org/10.3390/hematolrep17060060 - 7 Nov 2025
Cited by 1 | Viewed by 1401
Abstract
Background: The aim was to evaluate whether an artificial intelligence (AI)-based tool for the automated quantification of the total metabolic tumour volume (tMTV) in patients with Hodgkin lymphoma (HL) could support nuclear medicine specialists in lesion segmentation and thereby enhance inter-observer agreement. Methods: [...] Read more.
Background: The aim was to evaluate whether an artificial intelligence (AI)-based tool for the automated quantification of the total metabolic tumour volume (tMTV) in patients with Hodgkin lymphoma (HL) could support nuclear medicine specialists in lesion segmentation and thereby enhance inter-observer agreement. Methods: Forty-eight consecutive patients who underwent staging with [18F]FDG PET/CT were included. Eight invited specialists from different hospitals were asked to manually segment lesions for tMTV calculations in 12 cases without AI advice, and to use automated AI segmentation in a further 12 cases, with editing as required, i.e., segmenting/adjusting 24 cases each. Each case was segmented by two specialists manually and by two different specialists using the AI tool, allowing for the pairwise comparison of inter-observer variability. Results: The median difference between two specialists performing manual tMTV segmentations was 26 cm3 (IQR 10–86 cm3) corresponding to 23% (IQR 7–50%) of the median tMTV in the dataset, while the median difference between two specialists tMTV adjustments using AI segmentations was 12 cm3 (IQR 4–39 cm3) corresponding to 9% (IQR 2–21%) (p = 0.023). The median difference in tMTV between measurements with and without AI was 3.3 cm3, corresponding to 2.3% of the median tMTV. Conclusions: An automated AI-based tool can significantly increase agreement among specialists quantifying tMTV in HL patients staged with [18F]FDG PET/CT, without markedly changing the measurements. Full article
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15 pages, 516 KB  
Review
MECOM-Rearranged Acute Myeloid Leukemia: Pathobiology and Management Strategies
by Utsav Joshi and Rory M. Shallis
Hematol. Rep. 2025, 17(6), 59; https://doi.org/10.3390/hematolrep17060059 - 31 Oct 2025
Cited by 2 | Viewed by 4746
Abstract
Acute myeloid leukemia (AML) is an aggressive clonal hematopoietic malignancy, characterized by marked biological heterogeneity and variable clinical outcomes. Among its rarer genetic subsets is AML with rearrangements of the MDS1 and EVI1 complex locus (MECOM), occurring in fewer than 2% [...] Read more.
Acute myeloid leukemia (AML) is an aggressive clonal hematopoietic malignancy, characterized by marked biological heterogeneity and variable clinical outcomes. Among its rarer genetic subsets is AML with rearrangements of the MDS1 and EVI1 complex locus (MECOM), occurring in fewer than 2% of newly diagnosed cases. This review examines the biology and clinical significance of MECOM-rearranged AML, with a focus on its diverse mechanisms of leukemogenesis, including chromosomal inversion and translocation involving 3q26. We discuss how aberrant EVI1/MECOM activity alters gene expression networks and drives malignant transformation. Current therapeutic approaches—including intensive chemotherapy, hypomethylating agents in combination with venetoclax, and allogeneic stem cell transplantation—are evaluated with particular emphasis on inv(3) and other t(3q26) subtypes. Despite these treatment strategies, outcomes remain poor, underscoring the urgent need for novel, more effective therapies for this high-risk form of AML. Full article
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12 pages, 263 KB  
Review
Navigating the New Era in Myelodysplastic Neoplasms: A Review of Prognostic Implications of the IPSS-M Score and 2022 WHO Classification
by Mihai-Emilian Lapadat, Oana Stanca, Nicoleta Mariana Berbec, Cristina Negotei and Andrei Colita
Hematol. Rep. 2025, 17(6), 58; https://doi.org/10.3390/hematolrep17060058 - 30 Oct 2025
Cited by 4 | Viewed by 2370
Abstract
Myelodysplastic neoplasms represent a diverse group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, peripheral cytopenias, and an inherent risk of progression to acute myeloid leukemia. Accurate risk assessment and patient stratification are critical to optimizing therapeutic approaches and clinical outcomes. [...] Read more.
Myelodysplastic neoplasms represent a diverse group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, peripheral cytopenias, and an inherent risk of progression to acute myeloid leukemia. Accurate risk assessment and patient stratification are critical to optimizing therapeutic approaches and clinical outcomes. In 2022, significant advancements reshaped both the classification and prognostic stratification of MDSs. The revised WHO Classification introduced crucial genetically defined subtypes, particularly those involving biallelic TP53 inactivation and SF3B1 mutations, shifting the emphasis from traditional morphology-based criteria to molecular ones. Simultaneously, morphological subtypes such as hypoplastic and hyperfibrotic MDSs were established as distinct entities with unique prognostic implications. At the same time, the introduction of the International Molecular Prognostic Scoring System (IPSS-M) provided a more precise prognostic stratification by integrating comprehensive molecular data alongside traditional clinical and cytogenetic parameters. Several validation studies have confirmed IPSS-M’s superior discriminative power compared to previous models, notably IPSS-R, improving predictions regarding overall survival and leukemia transformation. Nevertheless, practical considerations regarding the widespread application of IPSS-M have emerged, including concerns over economic feasibility and accessibility of advanced molecular testing methods, such as extensive Next-Generation Sequencing panels. This review synthesizes the recent literature and critical studies validating these classification and prognostic updates, discussing their clinical impact, practical considerations, and implications for targeted therapeutic strategies. By focusing on molecular pathogenesis, the latest classification systems and prognostic models promise significant advances in patient-specific management, setting the stage for future innovations in treatment and improved patient outcomes. Full article
10 pages, 819 KB  
Article
Role of Daratumumab, Lenalidomide, and Dexamethasone in Transplantation-Eligible Patients with Multiple Myeloma After the Failure of Bortezomib-Based Induction Therapy
by Shun Ito, Takashi Hamada, Masaru Nakagawa, Takashi Ichinohe, Hironao Nukariya, Toshihide Endo, Kazuya Kurihara, Yuichi Takeuchi, Shimon Otake, Hiromichi Takahashi, Hideki Nakamura and Katsuhiro Miura
Hematol. Rep. 2025, 17(6), 57; https://doi.org/10.3390/hematolrep17060057 - 29 Oct 2025
Viewed by 2350
Abstract
Background/Objectives: The role of daratumumab, lenalidomide, and dexamethasone (DRd) in autologous stem cell transplantation (ASCT)-eligible patients with multiple myeloma (MM) after first-line bortezomib, cyclophosphamide, and dexamethasone (VCd) treatment is not yet established. Methods: We retrospectively evaluated ASCT-eligible patients with MM who received second-line [...] Read more.
Background/Objectives: The role of daratumumab, lenalidomide, and dexamethasone (DRd) in autologous stem cell transplantation (ASCT)-eligible patients with multiple myeloma (MM) after first-line bortezomib, cyclophosphamide, and dexamethasone (VCd) treatment is not yet established. Methods: We retrospectively evaluated ASCT-eligible patients with MM who received second-line therapy with DRd after initial induction therapy with VCd between 2017 and 2023 (salvage group). For comparison, patients who successfully underwent per-protocol treatment with VCd induction, followed by ASCT during the same period, were selected (control group). Results: Eight patients with a median age of 61 years (range, 36–68 years) were included in the salvage group. After a median of 5 DRd cycles, the best response was partial response (PR) in two patients (25%) and a very good partial response (VGPR) in six (75%). All patients underwent ASCT, resulting in PR in one (13%), VGPR in four (50%), and stringent complete response in three (38%). Measurable residual disease (MRD) assessed using multicolor flow cytometry was negative in four patients (50%). The controls included thirteen patients with a median age of 60 years (range, 44–64 years). While most patients in both groups received various post-ASCT therapies, the post-ASCT 2-year time to the next treatment rate was slightly better in the salvage group than in the control group (88% vs. 49%, p = 0.089). However, hypogammaglobulinemia was more common in the salvage group (75% vs. 15%, p = 0.018). Conclusions: This small case series suggests that DRd is promising for ASCT-eligible patients with MM after VCd failure. Full article
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20 pages, 1094 KB  
Review
The Gut Microbiome Role in Multiple Myeloma: Emerging Insights and Therapeutic Opportunities
by Mina Y. George, Nada K. Gamal, Daniel E. Mansour, Ademola C. Famurewa, Debalina Bose, Peter A. Messiha and Claudio Cerchione
Hematol. Rep. 2025, 17(6), 56; https://doi.org/10.3390/hematolrep17060056 - 27 Oct 2025
Cited by 6 | Viewed by 3800
Abstract
Multiple myeloma is a hematological cancer depicted by the proliferation of plasma cells within the bone marrow, causing immune dysfunction and other abnormalities. The gut microbiome, the microbial community in the gastrointestinal tract, was found to modulate systemic immunity, inflammation, and metabolism. Although [...] Read more.
Multiple myeloma is a hematological cancer depicted by the proliferation of plasma cells within the bone marrow, causing immune dysfunction and other abnormalities. The gut microbiome, the microbial community in the gastrointestinal tract, was found to modulate systemic immunity, inflammation, and metabolism. Although the interplay between gut microbiome and multiple myeloma has been found in recent research, there is a gap in knowledge linking the effect of the microbiome on the pathogenesis and treatment of multiple myeloma. The imbalance in the gut microbiome, dysbiosis, may influence multiple myeloma pathogenesis through immune modulation and inflammation. Certain microbial species have been associated with multiple myeloma progression, complications, and therapeutic responses to treatment. Moreover, microbiome-derived metabolites, short-chain fatty acids, can influence the immune circuits associated with multiple myeloma progression. Understanding the bidirectional relationship between multiple myeloma and gut microbiota may provide insights into enhanced treatment and the development of new microbiome-based interventions. The current review provides a comprehensive highlight of current evidence linking the gut microbiome with multiple myeloma, demonstrating its significant roles in the development, progression, and treatment of multiple myeloma. Additionally, it focuses on the therapeutic potential of modulating the gut microbiome as a novel adjunct strategy in multiple myeloma management. Full article
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14 pages, 447 KB  
Article
Outcomes for Primary Central Nervous System Lymphoma from a Single Institution
by Sruthi Dontu, Jacob Boccucci, Michael Chahin, Amany Keruakous, Anand Jillella, Jorge Cortes, Vamsi Kota, Locke Bryan and Ayushi Chauhan
Hematol. Rep. 2025, 17(6), 55; https://doi.org/10.3390/hematolrep17060055 - 24 Oct 2025
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Abstract
Background: Primary central nervous lymphoma (PCNSL) is a rare, aggressive, non-Hodgkin’s lymphoma. Outcomes are poor with standard induction of high-dose methotrexate (HD-MTX)-based regimens and consolidation. We present retrospective data from the Georgia Cancer Center. Methods: A single retrospective chart review was [...] Read more.
Background: Primary central nervous lymphoma (PCNSL) is a rare, aggressive, non-Hodgkin’s lymphoma. Outcomes are poor with standard induction of high-dose methotrexate (HD-MTX)-based regimens and consolidation. We present retrospective data from the Georgia Cancer Center. Methods: A single retrospective chart review was conducted on all PCNSL patients from 2013 to 2023 to assess for various factors influencing care. Results: Of a total of 38 PCNSL patients, 6 died and 2 were lost to follow-up prior to therapy initiation, leading to a total of 30 patients for analysis. The median age was 62.3 (21–82 years). One patient had HIV/AIDS. Two patients were on immunosuppression for either kidney transplant or multiple sclerosis (MS). The HIV and MS cases were Epstein-Barr Virus (EBV)-positive. Completion of ≥six cycles of induction was predictive of response. Conclusions: PCNSL remains an area of high unmet need. Recent studies have shown that HD-MTX-based therapy and autologous stem cell transplantation afterwards leads to improved outcomes regardless of age; however, non-relapse mortality is important to consider. Our data from a primarily elderly and sub-rural cohort reiterate the efficacy of combination chemoimmunotherapy and impact of induction cycle number on response, regardless of age. A multidisciplinary approach and targeted agent maintenance should be considered to improve outcomes in the elderly. Full article
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