Autoimmune haemolytic anaemia treated with multiple transfusions, immunosuppressive therapy, plasma exchange, and desferrioxamine.

Overview

Severe autoimmune haemolytic anaemia in a five year old boy was unaffected by treatment with prednisone and splenectomy, but subsided after combined immunosuppressive therapy and three plasma exchanges. Over five months, a total of 93 transfusions of concentrated erythrocytes was given (equal to 18.6 grams of iron or 1.1 g/kg BW). This resulted in severe iron overload with cardiac, hepatic, and pancreatic complications, together with growth-retardation. These complications disappeared after treatment with desferrioxamine and vitamin C, but despite a normal growth hormone response to glucagon the concentration of somatomedin in serum remained low. Treatment by plasma exchanges and immunosuppressive agents may therefore be of value in severe haemolytic anaemia refractory to corticosteroids and splenectomy. Iron chelating therapy should be considered if multiple transfusions result in iron overload.