ISSN: 2578-501X
Authors: Nidhi B*
β Thalassemia is one of the most prevalent autosomal disorders, which affects more than 4,00,000 new born per year worldwide. In India, the carrier rate of β thalassemia varies from 3-17%. The development of red blood cell antibodies (both allo-and auto) remains a major problem in thalassemia major patients. Red cell autoantibodies appear less frequently but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA) and result in difficulty in cross-matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs or alternative treatments including intravenous immunoglobulin (IVIg) and rituximab (antiCD20 monoclonal antibody). We describe a case of 19 months old male patient, who was admitted with diagnosis of thalassemia major and had received multiple transfusions from several centers. He developed hemolytic anemia and cell panel was suggestive of autoimmune hemolytic anemia. Finding a compatible blood unit for him was a major challenge. To maintain his hemoglobin level, least incompatible blood unit was issued under steroid cover. He required blood transfusion every 3-4 weeks and each time least incompatible blood was issued to maintain hemoglobin level. Oral steroids including rituximab were given post transfusion for 1 week.
Keywords: Autoimmunization; Multitransfused; Thalassemia