Hyperhaemolysis syndrome in haemoglobin E / beta thalassaemia responding to cyclophosphamide therapy

Abstract

Hyperhaemolysis syndrome (HS) is a rare condition where blood transfusions are followed by destruction of both donor and recipient red cells, in the absence of significant detectable red cell antibodies. This phenomenon is reported only in a few children and is extremely rare in thalassaemia syndromes. We report a case of HS in a child with low G6PD enzyme levels and Hb E beta thalassaemia, in whom intravenous immunoglobulin (IVIG), oral methylprednisolone and splenectomy failed but cyclophosphamide therapy resulted in a successful outcome.