Objective:To evaluate the rate of seropositivity to hepatitis B and C and Human Immunodeficiency Virus (HIV) infections among children with β-thalassemia major receiving multiple transfusions in Ahmedabad, India, compared with healthy controls.Materials and Methods:The study was performed during January 2007 to January 2009 on multi-transfused children suffering with β-thalassemia major registered in the Prathama Blood Centre, Ahmedabad; Jeevandeep hospital, Ahmedabad; and Red Cross Blood Centre, Ahmedabad, and investigated for the prevalence and development of transfusion-transmitted infections. Hepatitis B surface Antigen (HBsAg), anti-Hepatitis C Virus (HCV) Antibodies (Ab), and HIV Ab were checked using a fourth-generation Enzyme-Linked Immunosorbent Assay (ELISA). Positive tests were confirmed by western blots. Healthy blood donors were used for the control group.Results:Hepatitis B surface antigen, anti-HCV Ab, and HIV Ab were positive in one of 96 (1.04%; 95% Confidence Interval (CI) = 0.17–1.3), 24 of 96 (25%; 95% CI = 11.4–14.2), and one of 96 (1.04%; 95% CI = 0.12–1.3), respectively. The rate of anti-HCV Ab was significantly higher in multi-transfused children suffering with β-thalassemia major. In thalassemia patients, the rate of positive anti-HCV Ab was significantly higher than that for positive HBsAg (P<0.001) and HIV Ab (P<0.001).Conclusion:It is concluded that HCV is the current major problem in multi-transfused children with thalassemia major and more careful pretransfusion screening of blood for anti-HCV must be introduced in blood centers.

Our limited data suggest at least 70% of children with pulmonary emboli referred for hematology evaluation have antiphospholipid antibodies and coagulation regulatory protein abnormalities.

Life long red blood transfusion remains the main treatment for b thalassemia major patients. The development of alloantibodies complicates transfusion therapy in thalassemia patients. Alloimmunization to red cell antigens is one of the most important immunological transfusion reaction and causes delayed type of transfusion reaction. A prospective study was conducted from January 2007 to January 2010. This was a cohorts of 115 patients were selected from regular transfusion group and they were followed for two and half year. They were followed up for the effect of transfusion during study period. There was a decline in patient number from 115 to 96 due to mortality and transfer of patient. A total of 96 multiply transfused thalassemia patients were prospectively included in this study and three consecutive samples collected after every 6 months and investigated for the development of alloantibody to red cell antigens. Tests for antibody screening and identification were performed on preserved sample to investigate prevalence and development of red cell alloimmunization by standardized laboratory techniques by same person at Prathama Blood Centre. A total of 96 patients were included in the study. 63 patients were males and 33 females. A total of five single alloantibodies were formed in five patients out of them four (80 %) belonged to Kell blood group system and one (20 %) from Rh system. It was observed that two (1.92 %) of new thalassemia patients developed red cell alloantibodies during study period. Red cell alloimmunization should be kept in mind in the patients receiving multiple transfusions. In present study, alloimmunization rate was 5.21 %. Mean transfusion duration in these patients was 23.90 days, probably due to presence of alloantibody. RBC alloantibody detection on regular interval and corresponding antigen negative blood transfusion is strongly recommended in transfusion dependent thalassemia patients.

The normal to increased respiratory burst of intact cord blood neutrophils is the result of alterations to oxidase components: increased content of cytochrome b(558) in the plasma membrane and decreased levels of cytosolic components p47-phox, p67-phox, and p40-phox.

Expression of hematoregulatory cytokines such as interleukin-1 (IL-1) in response to cytotoxic chemotherapy hastens hematopoietic recovery, but may also potentiate myelotoxicity if myeloid progenitors enter cell cycle before drug clearance. In the present study, the ability of recombinant human IL-1 receptor antagonist (IL-1ra) to protect hematopoietic progenitors was studied in a murine model of cyclophosphamide (CPA)-induced myelotoxicity. CF-1 female mice received 200 mg/kg CPA and either 10 mg/kg IL-1ra or an equal volume of 0.05% human serum albumin (HSA) intraperitoneally (i.p.), followed 12 hours later by IL-1ra or HSA. CPA and IL-1ra increased absolute neutrophil counts (ANCs) at days 2 (P = .001) and 14 (P = .0025) after CPA. In IL-1ra-treated mice, colony-forming units granulocyte-macrophage (CFU-GM)/tibia were increased twofold and threefold at days 2 (P = .0047) and 7 (P = .023), respectively, whereas high proliferative potential colony-forming cells (HPP-CFC)/tibia were decreased twofold to threefold at 8 hours (P = .039) and 24 hours (P = .0033), but were approximately threefold higher than HSA-treated mice at day 7 after CPA. Coadministration of CPA and IL-1 enhanced myelotoxicity compared with mice injected with CPA and IL-1ra or HSA. In vivo, IL-1ra protected HPP-CFC, but not CFU-GM, from hydroxyurea suicide after a single dose of CPA, suggesting that IL-1ra inhibited cycling of HPP-CFC. In vitro, IL-1ra did not alter proliferation of CFU-GM, but inhibited IL-1-enhanced proliferation of HPP-CFC. These data suggest that IL-1ra acts as an indirect negative regulator of hematopoiesis and protects HPP-CFC from CPA, possibly by inhibiting IL-1-enhanced proliferation of early myeloid progenitors.