title: Resolution of alloimmunization and refractory autoimmune hemolytic anemia in a multi-transfused beta-thalassemia major patient.
abstract: Beta-thalassemia is one of the most prevalent autosomal disorders, which affect more than 400,000 newborn per year worldwide. In India, the carrier rate of beta-thalassemia varies from 3-17%. The overall rate of alloimmunization in thalassemia patients has been reported to be 5-30% in the world, which is mostly contributed by the alloimmunization to minor blood group antigen. Among Asians, the incidence of red cell alloimmunization is 22%. The recommended treatment for beta-thalassemia major is regular blood transfusion every 3 to 4 weeks. The development of anti-red cell antibodies (alloantibodies and/or autoantibodies) can significantly complicate transfusion therapy. Alloantibodies are commonly associated with red cell hemolysis. Red cell autoantibodies appear less frequently, but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA), and 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 (anti-CD20 monoclonal antibody).
authors.affiliation: Department of Transfusion Medicine, Armed Forces Medical College, Pune, Maharashtra, India.
authors.forename: Joseph
authors.initials: J
authors.lastname: Philip
authors.affiliation: Department of Transfusion Medicine, Armed Forces Medical College, Pune, Maharashtra, India.
authors.forename: Neelesh
authors.initials: N
authors.lastname: Jain
keywords: Thalassemia major; alloimmunization; auto immune hemolytic anemia