Transfusion Support and Red Cell Alloimmunisation in Thalassemia Patients

Authors: Dibyajyoti S

DOI: 10.23880/hij-16000123

Volume 2, Issue 1

Abstract

Thalassemias are the most common mono genetic disease worldwide. It has high frequency extending from the Mediterranean basin through the Middle East (Iran), India and Southeast Asia. In India, the carrier rate of Beta Thalassemia varies from 3-17%. There is complete or partial defect in synthesis of alpha or beta globin chain synthesis, accordingly named alpha or beta Thalassemia. β Thalassemia may be classified in to 4 categories. 1) Thalassemia major, Thalassemia intermedia, Thalassemia minor (trait), silent carrier. Thalassemia major patients usually present early and are transfusion dependent. Patients with β Thalassemia trait (Thalassemia minor) have elevated hemoglobin A2 on hemoglobin electrophoresis as well as mild anemia and microcytosis. They do not require red cell transfusion. Patients with β Thalassemia intermedia presents late with variable levels of anemia and is of the NonTransfusion-Dependent-Thalassemia (NTDT) phenotype. Due to the severity of their anemia or complications of their disease some patients will go on to chronic transfusions. Thalassemia carriers are asymptomatic & usually have no hematologic abnormalities.

Keywords: Thalassemia; Electrophoresis; Alloimmunisation

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