Refractory hyperhaemolysis in a patient with beta-thalassaemia major

We report the case of a 1-year-old girl with newly diagnosed beta -thalassa emia major. Following an initial blood transfusion with phenotypically matc hed blood, she developed a haemolytic anaemia which progressed with subsequ ent transfusions. The Direct Antiglobulin test (DAT) was strongly positive with C3d and weakl y with IgG. The only free antibodies detected were a weak anti-H and a weak cold auto-antibody, which did not exhibit a wide thermal range. The indire ct Donath-Landsteiner and Ham's tests were negative. There was no sustained clinical response to steroids, immunoglobulin infusi ons or splenectomy, An HLA identical sibling donor was available for alloge neic bone marrow transplantation (BMT) and the haemolysis resolved during t he immunosuppressive transplant conditioning. Such hyperhaemolysis without significant red cell alloantibodies has previo usly been reported in patients with sickle cell anaemia, but only rarely in patients with beta -thalassaemia major.