Osteoporosis in Thalassemia Major-Reflection in Indian ScenarioPaperback, 18 April 2019

Beta-thalassemia represents a group of recessively inherited hemoglobin disorders first described by Cooley and Lee and characterized by reduced synthesis of β-globin chain. Homozygous state results in severe anemia, which needs regular blood transfusion. Life expectancy of thalassemia patients extended dramatically, by combination of transfusion and chelation therapy in-turn it give rise to various complications severe hepatosplenomegaly, bone marrow expansion, growth retardation, congestive heart failure bone deformity or even death. Osteoporosis is common even in well-treated thalassemic patients. There is only handful of data reflecting the BMD status of India thalassemic patients. Dual energy X-ray absorptiometry (DXA) is an excellent non-invasive choice for repeated measurements of any temporal changes of BMD because of 1% precision rate and low radiation exposure. BMD is determined by variety of genetic, environmental factors, its inheritance is thought to be under polygenic control. There have been conflicting results so far and genetic susceptibility to osteoporosis isn't fully understood.