Indian Journal of Hematology and Blood Transfusion最新文献

Recommendations regarding hematopoietic stem cell donor medical suitability are generally expert opinions and include legal regulations. With this study, we aimed to determine the frequency of hematological abnormalities and the effect of these abnormalities on early complications in related hematopoietic stem cell donors. Adult donors of allogeneic related peripheral blood hematopoietic stem cell transplantations performed between 01.Jan.2011 and 01.Jan.2021 were included in this multicenter study retrospectively. There were 318 donors. Baseline anemia (7.5%), was the most frequently observed hematological abnormality and found to be an independent risk factor for the development of any degree of anemia on the day of apheresis (p = 0.003, OR 18.6, 95% CI 2.6-131.5). Clinical complications and other laboratory complications were not significantly related to baseline hematological variables in the multivariate analysis. Although baseline hematological abnormalities are common in donors, their impact on early donor complications seems controversial.

Sarcopenia, defined as a loss of skeletal muscle mass and/or function, has been associated with poor survival in various cancers. This study evaluates the prognostic impact of sarcopenia in multiple myeloma (MM) using the Psoas Muscle Index (PMI). A total of 181 MM patients diagnosed between 2014 and 2024 were retrospectively analyzed. Sarcopenia was assessed using PMI measurements at the L3 vertebra level, with gender-specific cut-off values determined by ROC analysis (442.63 mm²/m² for males and 308.85 mm²/m² for females). Sarcopenia was identified in 29.8% of patients. Sarcopenic patients were significantly older, had lower BMI, albumin, and hemoglobin levels, and were less likely to be eligible for transplantation. Multivariate analysis revealed that older age (OR:1.14, 95%CI:1.08-1.32, p = 0.014), hypoalbuminemia (OR:2.13, 95% CI: 1.11-4.12, p = 0.023), and low BMI (OR:2.52, 95%CI:1.83-5.23, p = 0.001) were independent risk factors for sarcopenia. Sarcopenia independently predicted worse PFS (HR:1.628, 95%CI:1.065-2.487, p = 0.024) and OS (HR:2.095, 95%CI:1.207-3.636, p = 0.009). Other independent risk factors for OS included LDH > ULN (HR:2.026, 95%CI:1.130-3.631, p = 0.018), transplant eligibility (HR:0.338, 95%CI:0.191-0.596, p < 0.001), and high cytogenetic risk (HR:1.912, 95%CI:1.004-3.640, p = 0.049). Subgroup analyses showed that sarcopenia significantly affected OS in transplant-ineligible patients (HR:0.501, 95%CI: 0.262-0.957, p = 0.03) but had no significant impact on survival in transplant-eligible patients. Sarcopenia analysis by using PMI in MM patients can be considered a cost-effective, simple, easily applicable, and practical method for daily routine. Sarcopenia independently predicts worse survival outcomes, particularly in transplant-ineligible patients. Larger prospective studies are warranted to validate these findings and explore integrating sarcopenia into frailty indices for MM.

Mantle Cell Lymphoma (MCL) is a rare subtype of non-Hodgkin lymphoma (NHL), accounting for 5-7% of all lymphomas. Currently, there is no standardized treatment option for older patients (> 65 years), and optimizing MCL treatment in this group remains a significant clinical challenge. In this multicenter retrospective study, we aimed to evaluate the outcomes of first-line treatments, overall survival (OS), progression-free survival (PFS), and factors influencing survival in patients aged 65 years and older diagnosed with MCL. Patients aged ≥ 65 years diagnosed with MCL between November 2016 and January 2024 in six centers were included in this study. A total of 77 patients were included in the study, with 60 males (77.9%) and 17 females (22.1%). The median age was 71 years (range: 65-89). At diagnosis, 96.1% of patients had Stage III-IV disease, and 36.4% had an ECOG performance score of 2-3. Based on the MIPI-c score, 48% of patients had high-risk disease. As induction chemotherapy, 44.2% received R-CHOP, 13% received RB, 13% received R-CVP, and 29.8% received alternating R-CHOP/R-DHA-platinum. Additionally, 19.5% of patients underwent ASCT, and 28.6% received Rituximab maintenance after induction therapy or ASCT. The overall response rate (ORR) was 71.4%. After a median follow-up of 32 months, the median PFS was 18 months, and the median OS was 42 months. In multivariate analysis, independent risk factors for inferior PFS were LDH > ULN (HR: 2.09, 95% CI: 1.05-4.16, p = 0.035), Ki67 ≥ 30% (HR: 2.90, 95% CI: 1.38-6.10, p = 0.005), blastoid variant (HR: 1.86, 95% CI: 1.09-5.12, p = 0.040), and maintenance treatment (HR: 0.31, 95% CI: 0.12-0.75, p = 0.010). Independent risk factors for inferior OS were the maintenance treatment (HR: 0.29, 95% CI: 0.09-0.94, p = 0.040) and POD24 (HR: 2.04, 95% CI: 1.06-4.19, p = 0.044). There remains no universally accepted standard treatment for older MCL patients, and the disease remains incurable with current treatment modalities. However, advancements in targeted therapies and the optimization of maintenance strategies hold promise for improving survival in older and unfit patients. Future studies focusing on incorporating targeted therapies into first-line treatment and identifying high-risk factors that influence survival will be crucial in tailoring effective and individualized treatment approaches for this population.

Data remain limited regarding thrombosis among Thai patient with multiple myeloma patients and who experience thromboembolic events face a higher mortality rate compared to those without such complications The primary objective is to determine the prevalence of symptomatic venous and arterial thrombosis in Thai multiple myeloma patients, with secondary objectives including identifying survival impact of thrombosis and associated factors of thrombosis. The study includes newly diagnosed multiple myeloma patients from the single site of tertiary care center in Thailand between 2018 and 2022. Of the 146 enrolled patients, The prevalence of symptomatic venous thromboembolism, arterial thrombosis, and both thromboses were 8.22%, 1.37%, and 0.68% respectively. No difference of overall survival between thrombotic and non-thrombotic groups, the 3 years overall survival rates among multiple myeloma patients with and without thrombotic events were 0.6 [95%CI: 0.37-0.97] and 0.63 [95%CI: 0.54-0.73] respectively. In multivariable logistic regression analysis thromboprophylaxis seemed to be associated with lower risk of thrombotic events in our patient population [OR: 0.05] without increase risk of bleeding. The prevalence of symptomatic venous thromboembolism and arterial thrombosis among Thai multiple myeloma patients was relatively low. Thromboprophylaxis is protective factor for thrombosis without increased risk of bleeding. There is no survival impact of thrombosis in multiple myeloma patient.

Objective: The study aims to screen pregnant women for hemoglobinopathy, offer prenatal testing for identified mutations and empower them to make informed reproductive decisions.

Method: Amplification-Refractory Mutation System Polymerase Chain Reaction (ARMS PCR) was used to detect common mutations for thalassemia and sickle cell disease, with HBB gene sequencing offered if no mutations were found. Prenatal testing was performed through Sanger sequencing.

Results: A total of 1149 pregnant women were screened, and 144 were found to be carriers for hemoglobinopathies. On mutation testing, 36.1% of women had the IVS1-5 (G > C) mutation, followed by CD 8/9 (+ G), IVS 1-1 (G > T), CD 41/42 (-CTTT), CD15 G > A, and a 619 bp deletion (23.6%, 11.1%, 7.6%, and 6.9%, respectively). Of the 144 carrier women, 24 couples were found to be at risk for having a baby with haemoglobinopathy, while 19 couples were already diagnosed to be carriers of hemoglobinopathies. Prenatal testing was done on 38 couples which revealed five fetuses with homozygous and compound heterozygous pathogenic variants of the HBB gene.

Conclusion: ARMS PCR is an effective method for identifying point mutations. Prenatal genetic counselling and diagnosis are crucial in mitigating severe hemoglobinopathies. It enables couples with knowledge to make informed decisions about their reproductive options.

Supplementary information: The online version contains supplementary material available at 10.1007/s12288-025-01967-3.

36-year-old lady with Primary Sjogren's Syndrome presented with progressive dyspnoea and dry cough since 6 months. On evaluation, there were multiple nodular cavitating lesions in lung on CT scan, which were hypermetabolic on FDG-PET. Biopsy was consistent with extra-nodal marginal zone B cell lymphoma of MALT. She was treated with Rituximab, Cyclophodphamide, Doxorubicin, Vincristine and Prednisolone based chemotherapy with good clinical and radiological response. Hence, lymphomas, including MALT lymphomas should be suspected when a Sjogren's patient presents with multiple pulmonary nodules.

Blasts with cup-like nuclear morphology (Fig. 1) are classically described in acute myeloid leukemia (AML) with normal karyotype and mutations in NPM1 and/or FLT3-ITD, often demonstrating monocytic differentiation [1, 2]. However, this morphology is rarely associated with B-lineage acute lymphoblastic leukemia (B-ALL). Here, we present a case of a 33-year-old female who presented with fever and menorrhagia. Peripheral blood smear (PS) revealed 80% blasts, several with distinctive cup-shaped nuclei, prompting initial consideration of AML. Flow cytometric immunophenotyping (FCI) demonstrated CALLA-negative B-ALL with aberrant CD15 expression (Fig. 3), a phenotype strongly associated with KMT2A rearrangements [3]. Karyotyping and fluorescence in situ hybridization (FISH) confirmed KMT2A rearrangement (Fig. 2) and excluded IKZF1 deletion, and karyogram confirmed a t(4;11)(q21;q23) with additional abnormalities (Fig. 4. Molecular testing for NPM1 and FLT3-ITD was negative. Additional lineage markers (myeloid and monocytic) were also negative, ruling out a mixed B/myeloid phenotype. This case adds to the limited literature reporting cup-like nuclear morphology in B-ALL, which is typically associated with varied cytogenetic abnormalities, including BCR::ABL1 fusion and hyperdiploidy [3, 4]. The findings emphasize the critical role of FCI and molecular studies in distinguishing AML from B-ALL or mixed phenotype acute leukemias.

To prepare a therapeutic dose of platelets in adults, 4-6 platelet concentrate (PC) from whole blood or one unit of apheresis are required. This study aimed to evaluate the effect of the preparation process of apheresis-PCs and pooled-PCs from platelet-rich plasma (PRP) on the platelet microparticles (PMP) count and platelet activation during storage. This study used four apheresis-PCs from the Haemonetics MCS + and four pooled-PCs. Six PRP-PCs were pooled and filtered to prepare pooled PRP-PC. The quality of the PCs was measured by platelet count, pH, swirling, P-selectin expression (as a marker of platelet activation), and PMP count on days 1, 3, and 5. WBC and RBC count on the first day and bacterial contamination on day 5. The platelet count in the pooled PRP-PCs was significantly lower on the fifth day compared to the first day. The PMP count was significantly higher on day 5 compared to day 1 for the pooled PRP-PCs, for the apheresis-PCs, the PMP count was higher on day 3 and day 5 compared to day 1. The WBC and RBC count in the apheresis-PCs was significantly lower. The P-selectin expression on the first day in the apheresis-PCs was significantly higher. The PMP count in the apheresis-PCs was significantly higher than the pooled PRP-PCs on day 3. The pooled PRP-PCs met standard quality parameters like apheresis-PCs. Pooled PRP-PCs showed lower platelet activation and PMP compared to apheresis-PCs in the early storage, indicating potential for clinical use.

Introduction of Post-Transplant Cyclophosphamide (PTCy) based immunosuppression in Haploidentical Hematopoietic Stem Cell Transplants (HSCT) has shown to reduce the incidence of Graft vs. Host Disease (GVHD). However, data on its use in HLA matched settings is lacking. We describe our experience using PTCY in pediatric patients undergoing matched donor HSCT. We retrospectively analysed data of 16 patients who underwent HLA-matched HSCT using PTCy from March 2022-July 2024 at our institute. Sixteen patients of median age-6 years (Range:1-17 years) were analysed. Indications of transplant were Thalassemia in 10, severe aplastic anemia in 5 and Congenital dyserythropoietic anemia in 1. Conditioning regimes used were Rabbit ATG-Thio-Flu-Cy-2 Gy TBI in 8 and Rabbit ATG-Thio-Treo-Flu-2 Gy TBI in 3 which was preceded by two cycles of pre-transplant immunosuppression (PTIS); Rabbit ATG-Flu-Cy-4 Gy TBI in 5 patients of aplastic anemia. PTCy, Mycophenolate mofetil and cyclosporine were used as GVHD prophylaxis. One patient had primary and another had CMV induced secondary graft failure. Three patients had grade I-II acute GVHD at median 32days post HSCT (Range: 28-140days). None of the patients had chronic GVHD. CMV reactivation occurred in 8 patients at a median + 21 days (Range: 14-35 days). Median follow up duration post HSCT was 473days (Range:85-808 days). 1 year- Event-free and 1 year-overall survival rates were 81.25% and 93.7% respectively. PTCy-based approach appears to be promising in matched related and unrelated donor transplants for benign hematological disorders.