ABC of Blood Transfusion in Patients with Thalassemia Major

Abstract

Among the hypochromic microcytic anemias, iron deficiency anemia, anemia of chronic inflammation, sideroblastic anemia, and thalassemia (homozygous or double heterozygous) become symptomatic in early infancy. Thalassemias are monogenetic hereditary disorders related to decrease in the synthesis of globin chains (alpha, beta, delta, and gamma). These are the most prevalent monogenic disorders in the world though mostly seen on the hemoglobinopathy belt. Patients with homozygous, double heterozygous condition present as microcytic hypochromic hemolytic anemia, but minimal hematologic findings might also be seen with heterozygous inheritance. In severe forms, hepatosplenomegaly, paleness, and jaundice usually develop in early life.