Study on effectiveness of transfusion program in thalassemia major patients receiving multiple blood transfusions at a transfusion centre in Western India

Abstract

Background:Children suffering from beta-thalassemia major require repeated blood transfusions which may be associated with dangers like iron overload and contraction of infections such as HIV, HCV, and HBsAg which ultimately curtail their life span. On the other hand, inadequate transfusions lead to severe anemia and general fatigue and debility.Materials and Methods:Data were obtained from 142 beta-thalassemia major patients aged 3 years or more receiving regular blood transfusions at a transfusion centre in Western India from 1 April 2009 to 30 June 2009. The clinical data and laboratory results were subsequently analyzed.Results:Of the 142 patients, 76 (53.5%) were undertransfused (mean Hb <10 gm%). 96 (67%) of the patients were taking some form of chelation therapy but out of them only 2 (2%) were adequately chelated (S. ferritin <1000 ng/ml). 5 (3.5%) of the patients were known diabetics on insulin therapy. 103 (72%) of the patients were retarded in terms of growth. The prevalence of transfusion-transmitted infections (TTIs) such as HCV, HIV, and HBsAg was respectively 45%, 2%, and 2%, with the prevalence of HCV being significantly more than the general population. The HCV prevalence showed positive correlation with the age of the patients and with the total no of blood transfusions received. As many as 15% (6 out of 40) children who were born on or after 2002 were HCV positive despite the blood they received being subjected to screening for HCV.Conclusions:The study suggests the need to step up the transfusions to achieve hemoglobin goal of 10 gm% (as per the moderate transfusion regimen) and also to institute urgent and effective chelation measures with the aim of keeping serum ferritin levels below 1000 ng/ml to avoid the systemic effects of iron overload. In addition, strict monitoring of the children for endocrinopathy and other systemic effects of iron overload should be done. Rigid implementation of quality control measures for the ELISA kits used to detect HCV in donor blood needs to be done urgently. Alternately, more sensitive and specific measures (like NAT testing) should be employed for detection of HCV. In the absence of a definitive cure accessible and available to all patients, strict implementation of the above suggested measures will go a long way in improving the quality (and quantity) of life in patients of beta-thalassemia major.

Iron overload in beta thalassemia major patients

“…It was reported that 12.5% of beta thalassemia major patients were with serum ferritin levels less than 1000 ng/ml in a study conducted in Bhopal, India [20]. However, a similar study conducted in Western India reported that only 2% of the patients were with serum ferritin level less than 1000 ng/ml [31].…”

A Cross-Sectional Study on Burden of Hepatitis C, Hepatitis B, HIV and Syphilis in Multi-Transfused Thalassemia Major Patients Reporting to a Government Hospital of Central India

“…The high prevalence of HCV in our study population is in concordance with many other studies around the world. The prevalence of TTI in multitranfused thalassemia patients has also varied as per the region, methods of donor screening and screening of blood units and quality systems adopted evident from Table 4 [ [12][13][14][15][16][17][18][19][20]. Previous studies in Iran have reported the prevalence of HCV in beta-thalassemia patient at a wide range of 7-64 %.…”

Consensus Statement of HCV Task Force of the Indian National Association for Study of the Liver (INASL). Part I: Status Report of HCV Infection in India

“…Other recent studies in multi-transfused thalassemia patients have shown HCV prevalence rates of 2% 74 to 15%. 75 This disparity may reflect differences in the nature of blood donor pool (voluntary, replacement or remunerated), prevalence of HCV infection among donors, type of test kits used for blood-bank screening, nature of care of thalassemia patients, association of high transfusion rate with better survival. 76 The INASL task force collected data from 5 centers around the country (THINK Foundation in collaboration with Children's Liver Foundation in Mumbai, Thalassemia and Sickle society, Hyderabad; DMC Hospital, Ludhiana; SGPGI, Lucknow; and the Thalassemia Welfare Association, Chennai).…”