When a transfusion in an emergency service is not really urgent: hyperhaemolysis syndrome in a child with sickle cell disease

Abstract

A 13-month-old boy with sickle cell disease (SCD) from Equatorial Guinea, who had recently arrived in Spain, presented with fever. He had suffered from malaria and had received a blood transfusion. Following physical examination and complementary tests, intravenous antibiotics and a red blood cell (RBC) transfusion were administered. Soon after a second transfusion 5 days later, the haemoglobin level fell below pretransfusion levels, together with reticulocytopenia, and haematuria-the so-called hyperhaemolysis syndrome-requiring intensive care and treatment with intravenous immunoglobulins and corticosteroids, with resolution of the complication. We want to emphasise the importance of suspecting this rare, though severe complication that can appear after any RBC transfusion especially in patients with SCD, as the clinical syndrome can simulate other more common complications of these patients and a further transfusion is contraindicated. There is no standardised treatment, but intravenous immunoglobulin and corticosteroids are usually effective.

Keywords: haematology (drugs and medicines); haematology (incl blood transfusion); paediatrics (drugs and medicines).

Figure 1

Progress during admission. Hb, haemoglobin; IVIG, intravenous immunoglobulin; MP, methylprednisolone; Retics, reticulocytes; Bt, blood transfusion.