Indian Journal of Hematology and Blood Transfusion最新文献

C reactive protein/albumin ratio (CAR) is considered as a predictor for prognosis in a variety of systemic disorders including malignancies. We aimed to investigate the potential impact of pretransplant CAR on transplant related complications and clinical outcomes in patients undergoing allogeneic hematopoietic stem cell transplantation (alloHSCT). A total of 430 alloHSCT recipients [median age: 44(18-72) years; male/female: 258/172] were included in this retrospective study. Median follow-up period was 761(1-5910) days. The study population was divided into two subgroups as low- (< 0.312) and high-CAR (≥ 0.312) [AUC: 0.61 (95%CI: 0.55-0.67); p < 0.001]. The probability of overall survival (OS) was significantly lower in CAR^high group (27.7% vs. 32.1%, p < 0.001). The frequency of posttransplant early complications including Cytomeglovirus reactivation (p = 0.04), sinusoidal obstruction syndrome (p = 0.042), sepsis (p = 0.004), nephrotoxicity (p = 0.011), dialysis requirement (p = 0.001) and acute GvHD (p = 0.026) were found to be significantly higher in CAR^high group. Pretransplant CAR was shown to have a significant impact on OS (p = 0.009) in multivariate analysis. Pretransplant CAR may be a feasible and cost effective marker to predict alloHSCT outcome for better management of early transplant related toxicity and complications.

There is a lack of data from India on the prevalence of anemia of chronic disease or inflammation. This was a prospective observational cross-sectional prevalence study. Anemic patients underwent investigations namely a complete blood count with peripheral smear, serum ferritin level, iron, total iron binding capacity, transferrin saturation, vitamin B12 and folic acid level, reticulocyte count and stool for occult blood. Other investigations were performed as required according to the patient's clinical profile. Three hundred fifty-five patients were enrolled. A total of 109 patients (30.7%) had anemia of chronic disease (ACD) (30.7%). Sixty-three/263 (24%) females had ACD compared to 46/95 (48.4%) males. ACD was four times more common in the age group 80 years and above (56.5%) compared to the age group 18 to 39 years (13.9%). Seventy-two (66%) patients had mild anemia, 19 patients (17%) had moderate anemia and 18 patients (16%) had severe anemia. Diabetes mellitus (44%), hypertension (39%) and chronic kidney disease (25%) were the commonest underlying morbidity. Thirty-six patients (33%) had no underlying comorbidity or cause. The prevalence of anemia of chronic disease increases with age. The majority of anemia of chronic disease patients have mild anemia.

Pericardial effusion is an infrequent but life-threatening complication that occurs after hematopoietic stem cell transplant (HSCT). The present study aimed to analyze the etiology and outcome of patients who developed the entity. A retrospective analysis of patients who underwent allogeneic HSCT and developed symptomatic pericardial effusion between 2017 and 2023 was performed. Of 749 patients who underwent HSCT, 22 (3%) developed symptomatic pericardial effusion. The median age was 85 months (range: 26-252). The commonest indication for HSCT was transfusion-dependent thalassemia, which was more common than in patients who did not develop pericardial effusion (82% vs. 56%, P = 0.016). All the patients who developed effusion received myeloablative conditioning. The mean duration from transplant to effusion diagnosis was 98 days (range: 22-195). Sinusoidal obstruction syndrome was observed in a higher proportion of patients who developed pericardial effusion, compared to those who did not (41% vs. 17%, P = 0.002). Fast breathing with/without dyspnea was the commonest clinical presentation, observed in 16 (73%) patients. There was evidence of cardiac tamponade in 9 (41%) patients. Calcineurin-inhibitor was withheld in 12 (54%) patients followed by a short course of glucocorticoid therapy. Sixteen (73%) patients had a complete resolution of the effusion after a mean duration of 12 days. Five patients succumbed to unrelated causes. Pericardial effusion is an infrequent but severe complication post-HSCT and tachypnea is the commonest presentation. Early identification of the etiology and appropriate management can lead to a complete resolution in most cases.

Supplementary information: The online version contains supplementary material available at 10.1007/s12288-024-01951-3.

To assessthe prevalence of the JAK2V617F mutation in polycythemia patients living at high altitude. This was a cross-sectional study, conducted at the National Institute of Blood Diseasesand Bone Marrow Transplantation (NIBD-BMT), KarachifromJuly 2022 to July 2023. A total of 132 patients with polycythemia (male, hemoglobin ≥ 16.5 g/dl; female, hemoglobin ≥ 16 g/dl) were enrolled in the study. The patients were categorized on the basis of high and low altitude living and further categorization of those living at high-altitude was done on the basis of JAK2 positive and negative status and characteristics. Blood sample of each patient was collected in aseptic environment and sent to laboratory for complete blood count. PCR was used to detect JAK2V617F mutation. Data was analyzed by SPSS version 24. Among 132 polycythemia patients, 94 (71.2%) were high altitude residents whereas 38(28.7%) resided at non-high altitude. Out of 94(71.2%) patients of high altitude, 49(52.1%) harbored the JAK2V617F mutation whereas no mutation was identified in 45(48%) patients. The difference in gender distribution, median age, Hb, TLC, platelet count and ANC was statistically significant in the JAK2 positive group (p < 0.05). Moreover, palpable spleen and presence of ischemic heart was also found to be statistically significant in JAK 2 positive patients. Other characteristics such as hypertension, diabetes mellitus, smoking, and other symptoms (headache, body aches, chest pain, itching, abdominal pain, blurring of vision, burning soles, and cough) showed no significant differences between the two groups (p > 0.05). JAK2V617F mutation was observed in 52.1% ofpolycythemiapatients living at high altitude.

Novel molecules including gene therapy are used to treat haemophilia. Little is known on patient perceptions about gene therapy. Present survey is to assess the knowledge and identify key educational gaps and concerns about gene therapy in Indian persons with haemophilia (PWH). An explorative, qualitative, closed questionnaire based survey was conducted recruiting adult PWH from 3 Centers in India. Data were collected through face-to-face surveys or the patients themselves filling the survey forms. Of the 415 complete questionnaires received from adult severe PWH, approximately half of the PWH felt that gene therapy is the best option for PWH, but only 31.2% showed their willingness to be a part of any gene therapy clinical trial, if available. About 57% of the PWH were unable to decide about participation in clinical trials and 11.7% of the participants would never consider receiving gene therapy. Major concerns were cost, side effects, duration of expression and rejection, infection and risk of liver cancer. The PWH expressed their desire for information on subjects like eligibility criteria, earlier trial data, and details on safety. The survey shows a low level of knowledge about gene therapy in PWH and identifies gaps in knowledge and a strong willingness to understand more on the potential risk: benefit profile of gene therapy.

Supplementary information: The online version contains supplementary material available at 10.1007/s12288-024-01945-1.

Acute Myeloid Leukemia (AML) is a heterogeneous hematologic malignancy, and up to 50% of cases are classified as cytogenetically normal AML (CNAML). The 2022 European Leukemia Net (ELN) guidelines identify Myelodysplasia Related(MR) gene mutations, such as ASXL1 and RUNX1, as adverse risk factors, particularly in patients treated with intensive chemotherapy. However, limited data exist regarding the prognostic impact of MR mutations in CN-AML patients managed with Azacytidine and Venetoclax (Aza+Ven) based induction. In the current study, consecutive patients with newly diagnosed CN- AML treated at one centre were classified as MR positive and negative on the basis of the presence and absence of MR mutations respectively. Clinical data, including patient demographics and treatment onsecutivet responses, were systematically recorded, and the cytogenetic and molecular data were analyzed to correlate these findings with clinical outcomes, such as complete remission rates and overall survival. Fifty three consecutive newly diagnosed AML patients treated with Aza+Ven were screened. Thirty two patients having CN-AML were included in the current analysis. The CR/CRi rates were comparable between MR mutated and MR unmutated groups (80% vs. 70%, p=0.5). The proportion of patients attaining MRD negativity after the first cycle was not significantly different [MR mutated (3/10, 30%) versus MR unmutated (7/22, 31%); (P = 0.95)] amongst the two groups. Median progression-free survival (PFS) and overall survival (OS) was not significantly different between MR mutated and MR unmutated groups (not reached vs. 12 months, p=0.437 and p=0.136,; HR-0.53, 0.22;p-0.45,0.17 respectively). Our findings, support that the presence of MR mutations may not adversely impact treatment outcomes when treated with Aza+Ven treatment regimen.

The aim of this study is to determine the incidence of a positive direct antiglobulin test (DAT) in a population of blood donors in Eastern India and to explore the impact of DAT positivity on donor health and transfusion services. We conducted a retrospective study on 89,402 blood donors over a seven-year period (2018-2024). Positive DAT results were detected using the column agglutination technique (CAT) following a difficulty in the cross-match, a positive weak D test on donor RhD typing, and a positive autocontrol encountered during donor antibody screening. Furthermore, DAT was subclassified, and donors exhibiting a DAT strength of ≥ 2 + were recalled and investigated. We found the DAT was positive in 54 donors (0.06%) with an overall incidence of 1:1,656. The DAT-positive donors presented predominantly as crossmatch incompatibility (85.2%). The majority of blood donors (96.3%) were IgG positive. Of the 39 donors who were evaluated, two were found to have autoimmune diseases. The remaining donors showed no signs of hemolysis and were in good health. The incidence of positive DAT results in eastern Indian blood donors is higher, leading to increasing non-utilization of collected blood units. There is a need for a national policy for managing DAT-positive donors in India.