Indian Journal of Hematology and Blood Transfusion最新文献

To diagnose rare coagulation factor deficiencies, study the varied clinical presentations along with their management and create a diagnostic algorithm in order to sensitize the treating physicians and diagnosticians for the effective management of patients. From the patients suspected of bleeding disorders, chief complaints and detailed bleeding history were recorded. A diagnostic algorithm was followed starting with first line tests for bleeding disorders which included prothrombin time, activated partial thrombin time, thrombin time, fibrinogen levels, liver function test and complete blood counts including platelet count. Isolated elevated PT or APTT were followed by mixing studies to confirm factor deficiency and inhibitor screen testing to rule out inhibitors. After ruling out common factor deficiencies (Haemophilia A and Haemophilia B), von Willebrand disease and platelet function defects, testing for RBD was done as per the diagnostic algorithm. A total of 66 patients were diagnosed with RBD. Male: female ratio was 1.1:1 with age varying from 1 month to 82 years, including 36 adults and 30 paediatric patients. Factor VII deficiency was most common with 22 cases (33.3%) followed by FX in 15 cases (22.7%), FXI in 12 cases (18.2%), FXIII deficiency in 11 cases (16.6%), FV in 3 cases (4.5%), FII in one case (1.5%) and combined FV + VIII comprised two cases (3%). Mucocutaneous bleeding was the commonest presentation. Moderate to severe bleeding episodes were most commonly observed in patients with FVII and FX deficiency with the mean age of presentation being 25.8 years and 22.9 years respectively. Mild to moderate bleeding episodes were observed in FV and XI deficiencies. The management of RBDs is usually based 'on demand'. The most common therapeutic product used was fresh frozen plasma in most of the cases, cryoprecipitate in patients with factor XIII deficiency mainly as a prophylactic therapy. Tranexamic acid was used in patients presenting with bleeding. Diagnosis and treatment of rare factor deficiencies is difficult. Knowledge of the clinical presentations, family history and availability of factor assay play a role in timely and correct diagnosis by using a diagnostic algorithm. In our study, we have provided the data from a tertiary referral centre of North India, Delhi which adds up to the limited data from our country.

Background: The study aimed to evaluate geriatric hematology patients in terms of geriatric syndromes, to provide the most appropriate individualized care and practices in the treatment processes, to access sufficient information about the patient and to contribute to the literature that geriatric hematology patients may be in the risk group in terms of geriatric syndromes.

Methods: In the study, the data were evaluated with urinary incontinence severity scale, mini nutritional analysis, Edmonton Frailty scale and an introductory information form created by the researchers.

Results: More than half of the geriatric individuals included in the study were diagnosed with leukemia. They had at least one chronic disease other than their hematological diseases, the average age was 70, more than half of them were frail, their MNA average was within the malnutrition risk limits, they were independent according to their average scores on Katz ADL. It was also determined that the participants were in the moderate incontinence range.

Conclusion: Elderly individuals with hematological malignancies should be considered for geriatric evaluation throughout treatment. Geriatric evaluation is important to prevent possible complications during treatment and improve quality of life.

Graft versus host disease (GVHD) occurs commonly after haematopoietic stem cell transplantation (HSCT). Cutaneous GVHD is a cause of significant morbidity. The use of conventional immunosuppressants leads to increase in the risk of infections and malignancy. Phototherapy is a relatively safe and efficacious alternative for treating chronic GVHD. We aimed to evaluate the effectiveness of psoralen + ultra-violet A (PUVA) therapy in cases of chronic GVHD. We included 10 cases of chronic GVHD, who had undergone HSCT at our centre. A retrospective, observational study analysis was carried out. Parameters like percentage of body surface area involved, percentage reduction in body surface area, number of phototherapy sessions, dose of oral steroid reduced and side effects were evaluated. Out of the total 10 patients, 7 (70%) had clinical features of lichenoid GVHD, while the other 3 (30%) cases had sclerodermatous type of GVHD. The mean time to develop chronic GVHD after bone marrow transplant was 8.25 months. The mean body surface area involved at the time of diagnosis was 53.1%. Seven patients (70%) responded to phototherapy. Five patients (50%) achieved complete remission, while two (20%) had partial remission. One patient (10%) was lost in follow up. One case (10%) died while on PUVA therapy due to relapse of leukaemia. One patient (10%) of lichenoid GVHD relapsed after completion of PUVA therapy. The average number of phototherapy sessions required were 34.6. The average percentage reduction in the dose of oral steroids before and after PUVA therapy was 89.12%. Other immunosuppressants were stopped after an average of 4.57 months in 8 patients. We hereby conclude that PUVA therapy is a much better alternative to the conventional immune suppressing agents for chronic GVHD.

Children with thalassemia often suffer from complications such as muscle weakness, bone fragility, fatigue and reduced physical endurance. As strengthening exercises have the potential to enhance physical function, increase independence, and improve overall quality of life for thalassemic child and Closed Kinetic Chain (CKC) exercises are proven to be beneficial due to their ability to engage multiple muscle groups simultaneously, leading to improved muscle strength and balance. Therefore the aim of the study was to determine the effect of closed kinetic chain exercises via telerehabilitation on muscle strength, balance, fatigue, and haemoglobin levels in Beta Thalassemia Major children This study involved 32 children aged 8-15 years with Beta thalassemia major, divided into experimental group (n = 16) receiving CKC exercises via telerehabilitation for 30-40-minutes, twice a week for 8 weeks, the control group (n = 16) receiving education on importance of being physically activity. Outcome measures were assessed at baseline, 4th week and 8th week which included balance using Bruininks-Oseretsky Test of Motor Proficiency (BOT-2); muscle strength, measured via hand-held dynamometer; fatigue levels by (PQL-MFS); and haemoglobin levels via the spectrophotometric method The experimental group showed significant improvements in balance (p < 0.001), muscle strength (p < 0.001), and fatigue reduction (p < 0.005), but no significant changes were observed on haemoglobin levels. An 8-week CKC exercise program delivered via telerehabilitation significantly enhanced balance, muscle strength, and reduced fatigue in children with Beta Thalassemia Major, though it did not affect haemoglobin levels.

Background: Ph-like ALL is a subtype of ALL with profile similar to Philadelphia-positive ALL but without BCR::ABL1 gene fusion and shows adverse clinical profile and inferior outcomes. This study aimed to identify Ph-like ALL using a simplified and cost-effective workflow in adolescent and young adult (AYA) instead of standardized ideal gene expression profiling. Due to the use of limited panel, the subgroup of B-ALL has been named as "B-ALL with overexpressed CRLF2, JAK2, ABL1".

Methods: We recruited AYA (13-40 years) patients with B-ALL. Patients were identified as "B-ALL with overexpressed CRLF2, JAK2, ABL1" if they had overexpression of any one of Cytokine receptor-like factor-2 (CRLF2) by flow cytometry (FCM), ABL1, or JAK2 by Reverse transcription polymerase chain reaction (RT-PCR).

Results: Of 100 patients, N = 14 (14%) were classified as Ph + ve (or BCR::ABL1 fusion present). Remaining N = 86 (86%) were classified into three groups: N = 41 (41%) Ph-ve, N = 29 (29%) "B-ALL with overexpressed CRLF2, JAK2, ABL1", and N = 16 (16%) unclassified (JAK2 and ABL1 negative, and CRLF2 expression unknown). During induction, 18/100 patients died, and 82 had end-therapy bone marrow studies with MRD data in 65 cases. CR was achieved in 75/100 (75%) cases. The CR was significantly lower (72%, p = 0.01) and MRD positivity was significantly higher (40%, p = 0.02) in "B-ALL with overexpressed CRLF2, JAK2, ABL1" than in other groups. For entire population, median follow up was 26 months, median EFS was 15.1 months and median OS was 15.9 months. For "B-ALL with overexpressed CRLF2, JAK2, ABL1", median EFS (1.5 months, p < 0.001) and median OS (5.1 months, p = 0.001) were significantly lower than the other three groups.

Conclusion: Using 3 markers representing "B-ALL with overexpressed CRLF2, JAK2, ABL1", we identified a group with poor outcomes. Conventional identification of Ph-like is expensive, requires expertise, and is difficult to implement in practice. Hence, this minimalistic approach may be further validated in future studies.

Supplementary information: The online version contains supplementary material available at 10.1007/s12288-025-01968-2.

Infection is a cause of high morbidity and mortality in myeloma. The aim of this study was to identify risk factors for the occurrence of infection in patients treated for multiple myeloma in the clinical hematology department at Dalal Jamm Hospital. A case-control study was conducted from January 1, 2005, to April 30, 2024. Each patient was matched with a control of the same sex and age ± 2 years. Patient diagnosed with myeloma according to International Myeloma Working Group criteria and who had an infection was considered a case, and controls were myeloma patients without infection. Data were analyzed using SPSS Statistics version 25 software. A p-value < 0.05 was considered significant. The Shapiro test was used to test for normality in the distribution of variables. Pearson correlation was used to determine the strength of association. One hundred and eight (108) cases and 80 controls were included, with a median age of 61.1 ± 9.67 years for cases and 62.15 ± 11.39 years for controls. The sex ratio was 1.25 and 1.05 in cases and controls, respectively. Infection was predominantly pulmonary (50%) and urinary (30%). Median survival was 1.2 years for cases and 2.6 years for controls. In multivariate analysis, risk factors for infection were a Charlson comorbidity score ≥ 1 (p = 0.006; OR = 2.39; CI: [1.247-4.599], positive CRP (p = 0.0040; [OR = 0.473; CI 95%: [0.231-0.966]), renal failure (p = 0.045; [OR = 2.263; CI95%: [0.210-24.342]) and type of treatment. The risk factors identified in our study were the presence of comorbidity, a positive CRP, the presence of renal failure and the treatment.

Background: Extracorporeal photopheresis (ECP) is widely used for graft-versus-host disease (GVHD), but manufacturer recommendations suggest maintaining a pre-procedure hematocrit (Hct) above 27%. This study evaluates the safety and efficacy of ECP performed at lower Hct levels.

Methods: We retrospectively analyzed 13 ECP procedures in three male patients with GVHD, comparing sessions performed at Hct < 26% to those at Hct > 27%. Procedural parameters, hemodynamic stability, and adverse events were assessed.

Results: All procedures at Hct < 26% were successfully completed without adverse reactions, vascular access issues, or significant hemodynamic changes. Average procedure duration was 97 min at Hct < 26% and 95 min at Hct > 27%, showing no significant impact on efficiency.

Conclusion: ECP at Hct < 26% is safe and effective, reducing the need for transfusion while maintaining procedural integrity. These findings challenge current Hct thresholds and support more flexible patient selection criteria.

Alloreactive KIR-B haplotypes mediate potent anti-tumor and anti-microbial effects and can improve the prognosis of haploidentical hematopoietic cell transplantation (HHCT) but there is paucity of this data in the Indian population. We aimed to evaluate the prevalence of KIR-B haplotypes and their alloreactivity in HHCT donors at our center. A total of 119 individuals (haploidentical donors, (n = 93); and patients, n = 26) from 26 families were included in this study. The KIR genotyping of the donors and HLA-B, as well as HLA-C genotyping of donor-recipient pairs, was done by polymerase chain reaction with sequence-specific primer genotyping assay. The KIR B-content score of donors was calculated using the online donor KIR B-content group calculator of the Immuno Polymorphism Database (IPD)(www.ebi.ac.uk/ipd/kir/donor_b_content). The alloreactivity of KIR was determined using an online KIR ligand matching calculator of IPD (www.ebi.ac.uk/ipd/kir/matching/ligand). Haploidentical donors were siblings and parents in 64% and 36% of cases, respectively. The prevalence of KIR-A (A/A) and KIR-B haplotype (B/A or B/B) in donors was 21% (20/93) and 79% (73/93), respectively. The KIR-B content score was 0 in 21% (20/93), 1-2 in 66% (61/93), and 3-4 in 13% (12/93) of donors. Accordingly, donors were classified as better/best in 77% (20/26) and neutral in 23% (6/26) of screened families. The KIR alloreactivity was absent in 44% (41/93) of donors while it was present in the recipient-versus-donor direction in 21% (20/93), the donor-versus-recipient direction in 23% (21/93) and both directions in 12% (11/93) of donors. Our study shows that over two-thirds of donors have KIR-B haplotype and over two-thirds of families have killer-B content based better/best donors but KIR alloreactivity in GvH direction in approximately one-fourth of donors highlights the feasibility of alloreactive KIR-B haplotype-based selection of suitable donors for HHCT.