A Prospective Study on Correlation of Grade and Pattern of Liver Siderosis with Amount of Transfusion in β Thalassemia Major Patients Undergoing Splenectomy for Assessment of Hepatic Iron Overload

Chronic hyper transfusion therapy in β thalassemia major patients is designed to maintain a hematocrit of at least 27-30% so that ineffective erythropoiesis is suppressed. Transfused RBCs, being taken up and degraded by RES/macrophages, results in overloading of recycled iron and excess iron saturates the binding capacity of Transferrin, thereby getting stored as hemosiderin in liver. The purpose of this study was to determine amount of transfusion and its correlation with grades and the patterns of liver siderosis to assess extent of iron overload and whether splenectomy needs to be adopted for a patient on the basis of amount of transfusion to prevent advancing liver siderosis. A total of 30 patients of β thalassemia major have been evaluated. Mean amount of transfusion for patients getting Grade 2, 3 and 4 liver siderosis were 31, 37 and 46 units respectively with a lone patient of grade 1 liver siderosis having been transfused 21 units of blood. Mean amount of transfusion required for appearance of HH and Mixed (HH; non-HH) patterns of liver siderosis were 38 and 48 units respectively. Correlation between amount of transfusion and grade of siderosis was calculated using Spearman's Rho Correlation coefficient and it showed statistically significant correlation between Total number of units transfused and grade of liver siderosis (Spearman's Rho value of 0.692 and p-value < 0.01). So, splenectomy should be done before a patient gets to higher grade and Mixed (HH; non-HH) pattern of liver siderosis and