Beta thalassemia in a tertiary care hospital with their chelation habit.

ABSTRACT

Beta-thalassemia which is one of the most common genetic disorders in Bangladesh encompasses a group of monogemic diseases that have reduced synthesis or no synthesis of one or more globin chain of haemoglobin. The defects involved are extremely heterogenous and give rise to a large phenotypic spectrum with patients that are almost asymptomatic to cases in which regular blood transfusions are required to sustain life. The aim of the study is to estimate the frequency of beta thalassemia among the blood recipients in the Day Care Centre of Transfusion Medicine Department of Mymensingh Medical College Hospital from July, 2009 to June, 2010 along with their chelation habit. Retrospective cross-sectional study with the sample size of 784, clinical and family data was collected and descriptive statistics were done in the Laboratory of Transfusion Medicine Department. Among the 784 subjects, the beta thalassemia was 521 (66.46%), among the thalassemic patients only 59(11.32%) take injection Dysferrioxamine (Dysferol), the iron chelating agent. Male are predominant than female (63.53%). Those of the affected patients 67.18% were in age group 6 to 20 years. Although beta thalassemia is one of the first monogemic diseases and represents a global health problem, only recently has the scientific community started to focus on the real molecular mechanisms that underline this disease, opening new & exciting therapeutic perspectives for thalassemic patients worldwide. It is concluded from our study that availability of blood transfusion facilities along with an orally and cheaper iron chelator is an urgent need to ensure the management of needy thalassemic patients in Bangladesh.