Indian Journal of Hematology and Blood Transfusion最新文献

Background

Thalassemia is the most frequent congenital cause of anemia globally, categorized by anomalous hemoglobin production. Lifelong PRBC transfusion with iron chelation therapy is the only therapeutic option available for the majority. Long-term recurrent blood transfusion has its hindrances like RBC alloimmunization. As there is inadequate data on alloimmunization in the pediatric thalassemia major population, our study aims to find out its prevalence and correlation with the clinical spectrum & transfusion profile.

Methodology

Alloantibody screening was done using 3 cell screening panel that includes antigens D, C, c, E, e, K, k, Fya, Fyb, Jka, Jkb, Lea, Leb, P1, M, N, S, s, Mia, Dia, and Xga. The presence of an unexpected antibody in the patient’s serum was considered a positive antibody screen for alloantibody. All samples were further examined to identify antibody specificity using 11 cell identification panels.

Results

6% (5/82) of the included patients demonstrated presence of one or more alloantibodies Alloimmunization was significantly greater in children having their first transfusion after one year of age [OR (95% CI) = 1.42(1.36-1.49), p = 0.02]; receiving > 12 transfusions per year [OR (95% CI) = 1.26(1.12–1.40), p = 0.03] and having > 150 ml/kg/year annual packed RBC consumption [OR (95%CI) = 1.13 (1.08–1.19), p = 0.05]. Total number of transfusions > 100 was also found to be positively associated with alloimmunization [OR (95% CI) = 1.22 (1.14–1.32), p = 0.04].

Conclusion

In the present study, alloimmunization was found to be 6% in multitransfused pediatric thalassemia patients. Our observation re-emphasizes the prerequisite for RBC antigen typing ahead of the first transfusion and early institution of transfusion therapy after diagnosis to decrease alloimmunization.

Excessive production of reactive oxygen species (ROS) leading to oxidative stress have been associated with many leukemias. In order to show whether there is a difference in ROS levels and total antioxidant power between BCR-ABL1 negative myeloproliferative neoplasms (MPNs) patients and controls, this study aims to evaluate the oxidant/antioxidant status in patients with MPN. 43 subjects with BCR-ABL1 negative MPNs and 40 healthy controls were included in this study. Oxidative stress was investigated by determination of total antioxidant power, catalase activity and concentrations of vitamins (A, C and E), malondialdehyde, hydroperoxides and carbonylated proteins. Oxygen Radical Absorbance Capacity (ORAC) was lower in BCR-ABL1 negative MPN patients compared with control group (0.40 ± 0.17 vs. 0.81 ± 0.06) AU (p˂0.01). Vitamins A (0.61 ± 0.19 vs. 0.82 ± 0.11) mol/L (p˂0.05), E (0.29 ± 0.08 vs. 1.10 ± 0.56) mol/L (p˂0.01) and C (19.22 ± 0,49 vs. 45.52 ± 0.36)µg/mL (p˂0.01) concentrations were lower in the same group compared to controls. No difference in catalase (CAT) activity between the BCR-ABL1 negative MPN and control groups was observed (p > 0.05). Higher malondialdehyde (MDA) plasma levels were found in BCR-ABL1 negative MPN patients compared to controls (2.97 ± 0.24 vs. 0.38 ± 0.18) mmol/L (p < 0.01). No significant differences were observed between BCR-ABL1 negative MPN and control groups in plasma hydroperoxide and carbonyl protein rates (p > 0.05; p > 0.05). BCR-ABL1 negative myeloproferative neoplasms are associated with dysregulation of redox balance of oxidant /antioxidant leading to an oxidative stress status.

Neutrophils on peripheral smear provide subtle findings or clues to the diagnosis of haematological disorders. The presence of toxic granules, hypersegmentation, pelgerhuet anamoly, green inclusions provide valuable information for clinicians. One of the rarer inclusions in neutrophils are bilirubin crystals. They are usually seen in children with hyperbilirubunemia and sepsis. We present a case of incidentally detected bilirubin crystals in neutrophils of a three day old child with elevated levels of serum bilirubin. Identification of these crystals should alert the clinician about the possible bilirubin toxicity and poor prognosis.

Introduction

Haematological malignancies (HM) comprise of nearly 10% of all cancers. In oncology, the Health- related quality of life (HRQOL) measure assesses the physical, social and mental health aspects of patients undergoing treatment. There is lack of Indian data on HRQOL in patients with haematological malignancies. The main objective of our study was to assess the parameters of quality of life in adult patients undergoing therapy for haematological malignancies.

Materials and methods

We conducted a cross-sectional study between 1st March 2023 and 31st July, 2023. Patients included for the study were newly diagnosed patients aged ≥ 18 years with haematological malignancy- (lymphoma, leukemia, and multiple myeloma) and undergoing therapy. Patients with relapsed/refractory disease, palliative care and those on follow up or observation were excluded. The primary objective of our study was to explore the parameters of quality of life in adult patients undergoing therapy for haematological malignancies. The secondary objective was to identify factors which are most influential in affecting the quality of life (QoL) in these subset of patients. European Organisation for Research and Treatment of Cancer (EORTC) QoL questionnaire (QLQ C 30 version 3.0) was used for QoL analysis.

Results

A total of 130 patients were eligible for evaluation. The median age of patients was 55 years (Range- 18–78 years). The proportion of males was 1.6:1. The total mean global health status score including health and QoL was 73.5 ± 24.4. On comparison with EORTC reference score for all cancer patients, the global health status functional scale in our study was higher. The highest symptom score was observed for fatigue (40.1 ± 26.1) and lowest for diarrhoea being 5.7 ± 14.7. A similar trend in the symptom scales was seen in the EORTC cohort When age groups were compared, means for global health and physical functioning score had statistically significant difference. Amongst symptom scales, fatigue scores were higher as the ages progressed.

Conclusion

Assessment of QoL in patients with haematological malignancies should be integrated into clinical practice, to understand treatment related morbidity and thereby helping in choosing optimal treatment strategy.

Background

Acute erythroid leukemia (AEL) accounts for 3% to 5% of AML patients. We present a case of AEL with whole exome sequencing (WES) data.

Case Report

A 25-year-old male presented with easy fatiguability and fever on & off for 6 months. Complete blood counts revealed anemia, leukocytosis and thrombocytopenia. Peripheral blood smear showed numerous nucleated RBCs ~600nRBCs/ 100WBCs and no blasts

Bone marrow aspirate smears were markedly hemodiluted. Bone marrow biopsy was hypercellular for age with marked erythroid hyperplasia and predominance of early erythroid precursors. On immunohistochemistry, Glycophorin was diffusely positive, E-cadherin highlighted the early erythroid precursors and CD34 was negative. Possibility of AEL was considered.

Total 77 AEL associated genes were taken out for functional association studies. Mutations already described in literature for AEL noted in this case were EPOR, JAK2, TP53, GATA 2, NPM1 and WT1. Novel mutations found in this case were ERCC6L2, PYGO1 and MYH9

The patient left the hospital against medical advice and expired within a month of diagnosis.

Conclusion

This case is interesting as AEL presented in a young male and PBS showed 600nRBCs/ 100 WBCs. Targeted sequencing can help to confirm the diagnosis of AEL especially in dilute marrows.

Background

Thalassemia is a chronic condition which has psychosocial impact on children and their caregivers. To ensure appropriate medical management and social support, it is important that these children are enrolled in a proper day care centre led by a haematologist or trained paediatrician. This study assesses the impact of dedicated thalassemia day care services in lives of patients with thalassemia.

Material and Methods

Retrospective analysis of qualitative and quantitative data of children in Thalassemia Day care centre (established in March 2020) of a Pediatric hospital in Mumbai, India was done. A team comprising of hematologist, counselor, staff nurses, social worker, porters and blood bank technician was appointed. A total of 88 children with Thalassemia who were initially taking treatment in general wards were enrolled. Transfusion guidelines to maintain pre-transfusion hemoglobin between 9 and 10.5 g/dl were prepared. Better hematocrit of blood, fresh blood and leucodepletion filters were provided to all. Iron overload status was checked by serum ferritin and T2* MRI periodically and chelation optimized. Steps to strengthen primary and secondary prevention of thalassemia were taken. NGOs were engaged to assist patients socially and financially.

Results

Thalassemia day care centre (TDC) started in March 2020 with 88 registrations and as of March 2023, we are supporting 157 patients. Baseline pre-transfusion Hb was available for only 64/88 (72%) and ferritin levels in 62/88 (70%) patients prior to TDC as rest were not following up with hematologist. Amongst 66 patients, pre transfusion hemoglobin improved from mean of 8.2 g/dl (6.5–10.6 g/dl) (pre TDC) to 9.5 g/dl (8–10.6 g/dl) (post TDC). No patient has acquired a blood transmitted viral infection in these 3 years. Mean serum ferritin levels were 3285 ng/ml amongst 63 patients pre TDC and 3870 ng/ml amongst 88 patients post 3 years of TDC. HLA typing camps helped us in identifying 14 sibling matches, out of which 9 underwent successful Bone marrow transplant. As primary prevention strategy, 201 anemic pregnant females were screened and 8 carrier mothers were identified. For secondary prevention, 15 couples underwent antenatal screening to prevent birth of a child with thalassemia. Team also ensured psychosocial well-being of families, which is reflected in the positive feedback given by patients post 3 years of day care. Seven CMEs or awareness programs have been conducted by the team to spread awareness.

Conclusion

With a dedicated centre, we are now able to provide appropriate healthcare to patients which has helped in improving their hemoglobin as well as iron overload status. Children and families now have a more friendly and comfortable environment for taking transfusions.

This multicenter retrospective study evaluated the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) on survival and safety in patients with relapsed/refractory (R/R) Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). A total of 110 patients with R/R HL or NHL who underwent allo-HSCT between July 2007 and October 2022 at 7 adult stem cell transplant centers were evaluated. Progression-free survival (PFS), graft versus host disease-free survival (GRFS), and overall survival (OS) were the primary endpoints, and NRM was the secondary endpoint. Forty-one (37.3%) of the total patients were diagnosed with HL, 69 (62.7%) with NHL. The median age at the time of allo-HCT was 39.5 years (16–67), of which 66 (60%) were male. The median follow-up was 67.5 ± 8.1 months, and the rates of 5-year OS, PFS, and GRFS were 38.4%, 37%, and 34.8%, respectively. On multivariate analysis, CR/PR disease status after allo-HCT was significantly associated with longer PFS (HR: 13.47, 95% CI: 5.80–31.26, p = 0.000) and OS (HR: 5.23, 95% CI: 2.93–9.34, p = 0.000). CR/PR disease status after allo-HCT (HR: 5.79, 95% CI: 3.22–10.40, p = 0.000) and grade 1–2 acute GvHD (HR: 2.33, 95% CI: 1.25–4.35, p = 0.008) were significantly associated with longer GRFS. The 5-year cumulative incidence of NRM was 24.8% (95% CI, 12.5–36.7). The most common conditioning regimen was reduced intensity. Transplant outcomes are not influenced by disease subtype. However, the achievement of a CR/PR response after allo-HCT significantly prolongs OS, PFS and GRFS. In addition, the presence of acute grade 1–2 GvHD was found to be another factor prolonging GRFS. These results support the feasibility of allo-HCT, especially in heavily treated patients.