Total management of thalassaemia major.

Abstract

Guidelines are suggested to clinicians entrusted with the care of thalassemic patients. Care should be taken in the differential diagnosis of thalassemia intermedia and major. Patients requiring maintenance transfusion do better in every way on a high transfusion scheme, but the detection and treatment of hypersplenism becomes especially important for patients on such schemes. Splenectomy reduces the blood requirement significantly and permanently in the majority of patients with .beta.+ thalassemia major. Prophylactic penicillin 125 mg twice daily should be given indefinitely after splenectomy. Long-term Fe-chelation therapy with i.m. desferrioxamine 500 mg-1.5 g daily according to weight, is safe and feasible and can bring patients into Fe balance at a high body Fe load. Treatment for any thalassemic patients with an acute illness, in addition to therapy indicated for the intercurrent disease is discussed. The immediate family should be screened for thalassemia trait, and genetic counselling offered to carriers.