Thalassemia major and intermedia in jammu and kashmir, India: a regional transfusion centre experience.

Abstract

Data on status of thalassemia and hemoglobinopathies from the extreme northern part of India is scarce. We investigated socio-demographic characteristics and management issues related to β-thalassemia in Jammu and Kashmir, India. Data from 96 thalassemia major and intermedia patients visiting the department of transfusion medicine for their transfusion needs was collected. Parameters recorded included age group, age at diagnosis, gender, religion, districts of the state they belonged to, family history of thalassemia, blood group, type of thalassemia (major/intermedia), total number of transfusions received and chelation therapy status. Thalassemia major patients comprised 92 (95.8%) and intermedia 4 (4.2%) of the cohort. Most cases were diagnosed in infancy or early childhood. The districts of Jammu and Rajouri together contributed 53% of the cases. Most patients were Hindu (76/96, 79.2%). A positive family history was most often obtained from Muslim patients (8/18, 44.4%). Only 50% cases were on iron chelation therapy. There is a need to come up with a national/local policy to manage disease in endemic areas and a policy formulated to help families and patients. Data such as ours may help in health management planning for thalassemic patients in this region.