Search Results (73)

Background: Treatment strategies for myelodysplastic syndromes (MDS) range from supportive care to disease-modifying therapies, depending on risk stratification and patient characteristics. Hypomethylating agents (HMAs) remain the standard treatment for higher-risk disease, whereas allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only potentially curative option. This study evaluated real-world survival outcomes and prognostic factors in patients with MDS treated with HMAs or allo-HSCT. Methods: A total of 79 patients with MDS who received azacitidine, decitabine, or allo-HSCT were retrospectively analyzed. Overall survival (OS) was evaluated using Kaplan–Meier and Cox proportional hazards analyses. To address potential immortal time bias, a prespecified 6-month landmark analysis was additionally performed. Results: Median OS was 14.9 months (95% CI, 9.9–20.0) with azacitidine, 10.0 months (95% CI, 6.8–13.2) with decitabine, and 48.0 months (95% CI, 19.9–76.1) after allo-HSCT. Patients undergoing allo-HSCT were significantly younger and had better ECOG performance status than those receiving HMAs. After adjustment for age, ECOG performance status, and IPSS-R risk category, treatment modality remained significantly associated with OS in the multivariable Cox regression model. In the prespecified 6-month landmark analysis, the survival advantage associated with allo-HSCT remained significant, although the adjusted association was attenuated. Conclusions: In this real-world cohort of actively treated patients with MDS, allo-HSCT was associated with longer overall survival than HMAs. Although this association persisted after adjustment for major clinical confounders and in a landmark analysis addressing immortal time bias, residual confounding related to treatment selection cannot be excluded. These findings should therefore be interpreted as an association rather than evidence of a causal treatment effect. Full article

Background: Multiple myeloma (MM) is a hematological malignancy characterized by systemic inflammation and frequent renal involvement. The C-reactive protein-to-albumin ratio (CAR) has emerged as a potential inflammation-based biomarker; however, its clinical significance in MM remains incompletely understood. Methods: This retrospective single-center study included 62 patients with MM. Clinical and laboratory data at diagnosis were collected, and CAR was calculated as the ratio of CRP to serum albumin. Patients were stratified into low and high CAR groups based on the median value (0.13). Survival outcomes were assessed using the Kaplan–Meier method. Results: The mean age was 65.9 ± 9.9 years, and 54.8% of patients were male. Higher CAR levels were associated with shorter overall survival (123 vs. 89 months, p = 0.32) and progression-free survival (60 vs. 36 months, p = 0.16), although these differences did not reach statistical significance. An unexpected finding was that the requirement for dialysis was higher in the low-CAR group (21.2% vs. 3.4%, p = 0.04). Other clinical and laboratory parameters were comparable between groups. Conclusions: CAR may reflect systemic inflammatory status in patients with MM; however, its prognostic value for survival remains uncertain in this cohort. The observed findings, particularly regarding renal outcomes, should be interpreted with caution. Larger prospective studies are needed to clarify the clinical utility of CAR in MM. Full article

Background: In hematologic malignancies, treatment allocation and outcome prediction are traditionally driven by clinical and biological parameters. However, growing evidence suggests that non-clinical factors—such as psychosocial context, caregiver availability, organizational support, and digital health integration—play a pivotal role in patients’ ability to tolerate and adhere to complex therapeutic pathways. The concept of “resilience” may offer a more comprehensive framework to capture this multidimensional readiness to treatment. Methods: We conducted a narrative review of the literature focusing on patient and caregiver resilience in hematologic settings. PubMed, Scopus, and Web of Science were searched for studies published in English over the last 15 years, addressing clinical, psychosocial, organizational, and contextual determinants influencing treatment tolerance, continuity of care, and outcomes in hematology. Results: the literature highlights resilience as a dynamic construct shaped by clinical fitness, psychological resources, caregiver competence, social and family context, healthcare system organization, and access to supportive technologies such as telemedicine. Several domains emerged as recurrent determinants of resilience, yet no standardized, integrated assessment tool is currently available in routine hematologic practice. Conclusions: Resilience in hematology should be reframed as a multidimensional, context-dependent construct extending beyond traditional clinical fitness. Incorporating resilience-oriented assessment into clinical workflows may improve treatment personalization, optimize resource allocation, and enhance patient- and caregiver-centered care. Future research should focus on developing pragmatic, clinically applicable tools to operationalize resilience in real-world hematologic settings. Full article

Introduction: The concomitant occurrence of myeloproliferative neoplasms (MPNs) and plasma cell dyscrasias is rare and presents significant diagnostic challenges. Accurate distinction between overlapping features is essential, particularly when bone marrow fibrosis (BMF) is present. Case Description: We report a 57-year-old female, with a 10-year history of thrombocytosis managed with antiplatelet therapy, who presented with anemia and severe lumbar pain. Bone marrow biopsy revealed marked fibrosis, and imaging revealed multiple vertebral lesions. Diagnostic workup identified features consistent with myelofibrosis (MF) and coexisting IgG-Kappa multiple myeloma (MM). Although the patient initially fulfilled WHO criteria for MF, the rapid resolution of fibrosis following first-line plasma-cell-directed therapy suggested a secondary, cytokine-mediated process rather than a true concomitant MPN. Conclusions: This case highlights the importance of an integrated diagnostic approach in patients with overlapping features of hematologic malignancies. Differentiating between MM-associated fibrosis and true concurrent MPN and MM is critical, as misclassification may alter both prognosis and therapeutic strategy. In triple-negative cases, the histologic response to plasma-cell-directed therapy can serve as a key discriminating criterion. Awareness of the potential association between MM with fibrosis and extramedullary disease is also essential for clinical management. This case underscores the importance of an integrated diagnostic approach in patients with overlapping hematologic features. Full article

Background: The associations between mean corpuscular volume (MCV) and leukocyte-derived ratios and chronological aging remain poorly understood. We aimed to evaluate the association between MCV and leukocyte-derived ratios and chronological age in U.S. adults. Methods: This cross-sectional study used data from the National Health and Nutrition Examination Survey (NHANES, n = 9259) and the Health and Aging Brain Study–Health Disparities (HABS-HD, n = 770). Participants were aged ≥20 years, and individuals with hemoglobin levels outside 12–18 g/dL or C-reactive protein (CRP) > 1 mg/dL were excluded. Associations between age and MCV, neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), neutrophil-to-monocyte ratio (NMR), and platelet-to-white blood cell ratio (PWR) were examined using quartile comparisons and multivariable linear regression adjusted for sex, race/ethnicity, CRP, and red cell folate and hemoglobin. Results: In both cohorts, higher quartiles of MCV, NLR, and MLR were associated with older mean age. In adjusted marker-specific models, MCV, NLR, and MLR were each positively associated with chronological age. In composite models, MCV remained independently associated with older age in both NHANES (β = 0.646, 95% CI 0.567–0.725) and HABS–HD (β = 0.300, 95% CI 0.156–0.445). Conclusions: MCV and selected leukocyte-derived ratios are significantly associated with chronological age across two U.S. cohorts, with MCV showing the most consistent independent association. Full article

Blastic plasmocytoid dendritic cell neoplasm (BPDCN) is a rare myeloid malignancy, characterized by the involvement of multiple organs, including the skin, bone marrow and blood, lymph nodes and the central nervous system. According to tumor location, the disease is classified as skin-only, systemic-only, and skin and systemic. The cutaneous manifestations of disease are typical and are represented by violaceous single tumors or multiple plaques present in sun-exposed cutaneous areas. BPDCN is issued from the malignant transformation of dendritic cell progenitors and is diagnosed using the classical immunophenotypes CD123, CD4 and CD56 in addition to specific membrane markers of plasmocytoid dendritic cells. BPDCN is an aggressive disease and is associated with a short survival. Upfront therapies involve either chemotherapy regimens in fit patients and CD123-targeted therapies, including interleukin-3 conjugated with diphtheria toxin (Tagraxofusp, SL-401), or Pivekimab sunirine, an anti-IL-3R-drug conjugate, for both fit and unfit patients. Targeted treatments limit the toxicities of chemotherapy and allow the bridging of a consistent proportion of patients to hematopoietic stem cell transplantation, the only treatment associated with potential long-term survival. Full article

Background: The co-existence of hemophilia and cancer presents one of the most complex clinical scenarios, demanding individualised therapeutic planning to balance oncologic efficacy and hemostatic safety. This study evaluated the ability of two Large Language Models (LLMs)—ChatGPT (GPT-4) and Microsoft Copilot (GPT-4–based)—to generate clinically appropriate recommendations for real cases of hemophilia with concurrent malignancy. Methods: Six consecutive adult cases of hemophilia and cancer, managed at the Hemophilia Centre of Padua, Italy, were selected for evaluation. Identical structured prompts were submitted to both LLMs. Two independent expert clinicians rated the model outputs across five domains (Decision/Rationale, Strategy, Selected Drug, Regimen, and Assessment) using a four-level ordinal scale. Results: LLMs demonstrated uneven performances. Outputs were consistently rated as highly reliable in domains involving high-level synthesis, such as Assessment and Strategy. However, substantial limitations were observed in the clinically demanding domains of Selected Drug and Regimen. Critically, in the Selected Drug domain, there was complete agreement between the two expert raters for neither system. This severe lack of concordance signifies that clinicians assigned different adequacy ratings to the same output in every case, reflecting ambiguity, lack of specificity, and inconsistent clinical interpretability of the drug-related information provided by LLMs. Conclusions: While LLMs possess the capacity for high-level reasoning and strategic planning, their inability to translate principles into precise, consistent, and clinically interpretable therapeutic plans—particularly regarding drug selection and treatment regimens—is a significant constraint. These deficiencies, highlighted by the minimal expert concordance in critical domains, necessitate rigorous clinical validation before the responsible integration of LLMs into the management of this uniquely vulnerable patient population. Full article

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of peripheral T-cell lymphoma (PTCL) and is frequently associated with autoimmune phenomena. Clinically, AITL shows an aggressive disease course and poor prognosis with currently available treatment strategies. We here report the case of a 64-year-old female patient who was diagnosed with AITL and showed a complicated clinical course due to concurrent immune-mediated thrombotic thrombocytopenic purpura (iTTP). To our knowledge, the presented case highlights a previously unreported association of both conditions. Treatment, including chemotherapy and iTTP-directed treatments, resulted in rapid clinical improvement and sustained remission of both the AITL and the concurrent iTTP. In AITL, transformed T-follicular helper cells (TFHs) are particularly thought to mediate hypersecretion of cytokines and excessive autoantibody production. Immunological disturbances to large parts mediated through these transformed TFHs are thought to trigger autoimmune conditions, as seen with iTTP in this patient. At 36 months post-treatment, the patient remains in complete remission for both AITL and iTTP. This case highlights the complex immunopathological relationship between AITL and autoimmune disorders possibly impeding diagnosis and treatment in a timely manner. Full article

Background: Sickle cell disease (SCD) is a hereditary blood disorder marked by the production of abnormally shaped, rigid red blood cells that obstruct blood flow, resulting in pain, organ damage, and increased infection risk. SCD poses a significant public health challenge in Nigeria, which has the highest global burden, with about 150,000 affected children born annually. The high prevalence is exacerbated by limited healthcare infrastructure, low public awareness, and socio-economic barriers, making effective disease management difficult. Understanding the knowledge of home-based caregivers is essential to identify gaps that may impact care quality. This study was performed within the African Research and Innovative Initiative for Sickle Cell Education (ARISE, EC GA No 824021) project to develop best practice in the clinical management of SCD. Aim: This study explores the knowledge, experiences, and educational needs of home-based caregivers of children with SCD attending the Paediatric Haematology Clinic, ABUTH, Zaria. Methods: A qualitative case study design was used, involving in-depth interviews with ten purposively selected caregivers. Interviews were conducted in Hausa, transcribed, and translated into English. Thematic analysis was performed. Results: Four themes emerged: 1. Understanding of SCD aetiology 2. Knowledge of symptoms 3. Awareness of complications and 4. Knowledge of SCD type. Conclusions: Home-based caregivers had limited knowledge of the genetic basis of the disease, but possess some knowledge of SCD key symptoms, enabling basic disease management and healthcare seeking. However, there is a need to enhance caregiver education to improve care quality and health-seeking behaviour for children with SCD. Full article

Background/Objectives: Tyrosine kinase inhibitors (TKIs) have transformed the treatment of chronic myeloid leukemia (CML), yet emerging evidence indicates an increased risk of vascular adverse events, particularly peripheral artery disease (PAD). Reliable biomarkers for early detection of TKI-related vascular toxicity are still lacking. Methods: A cross-sectional study was conducted on 78 patients with chronic-phase CML treated at Dr. Soetomo General Hospital, Surabaya. PAD was confirmed using ankle–brachial index. Serum oxidized low-density lipoprotein (OxLDL) and interleukin-10 (IL-10) levels were measured using ELISA. Results: PAD was detected in 20% of subjects. The PAD group showed significantly higher OxLDL, lower IL-10, and a markedly elevated OxLDL/IL-10 ratio (all p < 0.001). OxLDL remained independently associated with PAD after adjustment (adjusted OR = 1.132, 95% CI 1.020–1.255, p = 0.019). OxLDL/IL-10 ratio yielded a good diagnostic value (sensitivity 87.5% and specificity of 88.7%). Conclusions: Elevated OxLDL and an increased OxLDL/IL-10 ratio are associated with PAD in CML patients receiving TKI therapy and demonstrated a good diagnostic performance for early detection of TKI-induced vascular toxicity. Full article

Introduction: Ropeginterferon alfa-2b is an emerging treatment for polycythemia vera, with growing interest in its application for essential thrombocythemia and early myelofibrosis due to its extended dosing intervals and favorable tolerability profile. However, real-world evidence regarding its dosing strategies and titration practices remains limited. Objective: This study examined seven younger patients, all under 60 years of age, who were treated with ropeginterferon alfa-2b. Materials and Methods: This study is a retrospective medical records review of consecutive patients from seven hospitals. Treatment was initiated at a dose of 250 micrograms, with a maintenance dose of 500 micrograms. Results: The regimen demonstrated good safety and tolerability in this real-world setting. Hematological responses were observed, along with a meaningful reduction in JAK2V617F variant allele frequency across the patient cohort. Conclusions: These findings show that the use of high-initial-dose accelerated titration (HIDAT) regimen of ropeginterferon alfa-2b is a safe and effective treatment option for younger patients with myeloproliferative neoplasms. Full article

Background: The emergence of therapy-related myelodysplastic syndrome (t-MN) after autologous stem cell transplantation (ASCT) is well documented. However, with the growing use of chimeric antigen receptor (CAR) T-cell therapy for relapsed/refractory B-cell malignancies, concerns about secondary myeloid neoplasms, particularly MN, have arisen. The mechanisms and cytogenetic features associated with post-CAR-T MN, especially chromosome 7 abnormalities, remain underexplored. Objectives: To compare the incidence, timing, and cytogenetic characteristics of MN developing after CAR-T-cell therapy versus ASCT, and to evaluate the potential association between CAR-T therapy, persistent cytopenias, and these specific alterations. Study Design: This was a retrospective, single-center study of 275 patients with B-cell malignancies treated between 2015 and 2024 at Hospital Universitario Central de Asturias (Spain). Of these, 259 patients underwent ASCT and 16 received CAR-T-cell therapy (axicabtageneciloleucel n = 13, tisagenlecleucel n = 2, brexucabtageneautoleucel n = 1). Clinical, cytogenetic, and laboratory data were collected and analyzed. Incidence rates were compared using Fisher’s exact test, and time-to-event outcomes was evaluated using the Mann–Whitney U test (given the small number of events). Statistical significance was set at p < 0.05. Results: Myeloid neoplasms were diagnosed in 3 of 259 ASCT patients (1.15%) and in 2 of 16 CAR-T-cell patients (12.5%) (p = 0.03). The median time to myeloid neoplasm diagnosis was numerically shorter in the CAR-T group (15.5 vs. 69 months, p = 0.096). All post-CAR-T cases presented persistent cytopenias and cytokine release syndrome (CRS). Cytogenetic analyses revealed de novo monosomy 7 and 7q deletion in both CAR-T-related cases, whereas no chromosome 7 abnormalities were detected in ASCT-related cases. Pre-treatment samples did not show these abnormalities, although limitations in the sensitivity of the assays preclude the definitive exclusion of minor pre-existing clones. Both affected CAR-T patients had prolonged CAR-T cell persistence and required transfusional support due to hematologic toxicity. One patient was diagnosed with high-risk MN with 5q and 7q deletion and the other with Clonal Cytopenia of Uncertain Significance (CCUS) with monosomy 7. Conclusions: CAR-T-cell therapy was associated with a significantly higher and earlier incidence of myeloid neoplasms compared to ASCT in this cohort. The development of post-CAR-T myeloid neoplasm was characterized by persistent cytopenias, prolonged CAR-T cell persistence, and de novo chromosome 7 alterations. While the small sample size necessitates cautious interpretation, these findings may suggest a distinct pathogenesis potentially linked to inflammation, immune toxicity, or the expansion of pre-existing clones. This highlights the need for long-term hematologic monitoring and evaluation for clonal hematopoiesis prior to CAR-T-cell therapy, especially in heavily pretreated patients. Full article

Background/Objectives: Understanding blood group antigen distribution is essential for transfusion safety and preventing alloimmunization in transfused patients. The ABO, RH1, and Kell blood group systems are among the most clinically significant due to their high immunogenic potential and their role in hemolytic transfusion reactions and hemolytic disease of the newborn. Despite their clinical significance, data on the phenotypic frequency of these samples in southern Chile are limited. This study aimed to identify the distribution of ABO, RH1, and Kell blood group systems among blood donors at the Centro de Sangre Concepción, adding regional data to the national transfusion medicine records. Methods: A retrospective, descriptive analysis was conducted using data from 59,318 blood donations collected in 2024 by the Concepción Blood Center, part of the Southern Transfusion Medicine Macronetwork in Chile. Blood typing for the ABO, RH1, and Kell antigen (KEL1) typing was performed in accordance with national regulations established by the Ministry of Health (MINSAL). Results: Blood group O was the most frequent (61.3%), followed by A (27.8%), B (9.0%), and AB (1.9%). RH1 positivity was observed in 94.47% of donors, and Kell positivity in 4.24%. The distribution of Kell phenotypes was comparable between men (4.38%) and women (4.11%), with the highest frequency in donors aged 27–52 years. Conclusions: The phenotypic distribution observed reflects national patterns and shows the genetic makeup of southern Chile. The low but important prevalence of Kell-positive donors emphasizes the need for systematic Kell antigen screening to prevent alloimmunization and improve transfusion safety. Full article

Background: Malignant lymphomas are among the most common hematological neoplasms and include a heterogeneous group of entities characterized by distinct morphology, immunophenotype, genetics, and clinical features. Recent advances in molecular diagnostics have significantly improved our understanding of the genetic lesions and mechanisms underlying lymphomagenesis. Methods: This review summarizes key developments in molecular pathology relevant to B-cell lymphomas, including updates from the World Health Organization classification and recent progress in genomic, immunophenotypic, and clinical assessment. We highlight findings from next-generation sequencing studies and other molecular approaches used in routine and research settings. Results: Many molecular alterations are now routinely incorporated into diagnostic criteria and influence risk stratification, prognosis, and treatment selection. Although not all lesions are evaluated in everyday clinical practice, several changes have demonstrated prognostic significance and therapeutic relevance. Molecular subclassification has refined our ability to predict clinical behavior and response to targeted therapies. Conclusions: Advances in molecular diagnostics continue to reshape the clinical approach to lymphomas. Improved classification, better identification of therapeutic targets, and more accurate prognostic tools collectively enhance personalized treatment strategies. As a result, molecular tools increasingly guide clinical decision-making and contribute to improved outcomes in patients with B-cell lymphomas. Full article

Background/Objectives: Complete blood count (CBC)-derived markers such as the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and lymphocyte-to-monocyte ratio (LMR) have gained increasing attention as accessible indicators of systemic inflammation. These parameters, calculated from routine blood tests, are widely available in clinical settings and are potentially relevant for a variety of chronic diseases. This review aims to explore current evidence and highlight potential future directions regarding the use of hematologic inflammatory biomarkers in chronic disease. Methods: We performed an extensive literature search on PubMed to identify full-text original studies published in the past five years, focused on investigating the clinical applications of hematologic inflammatory markers in chronic conditions. Results: CBC-derived inflammatory markers have been studied in a wide range of chronic diseases, including autoimmune diseases, metabolic disorders, chronic kidney disease, chronic infections, psychiatric diseases, and other conditions. These markers have been evaluated for multiple clinical purposes, such as aiding diagnosis, monitoring disease status, assessing disease activity, disease subtype characterization, predicting prognosis, and evaluating associations with disease outcomes. Conclusions: As chronic diseases affect millions of individuals globally, placing a burden for the healthcare system, patients, and their families, simple and cost-efficient tools like CBC-derived inflammatory markers have the potential to improve clinical case management. Full article