AGRANULOCYTOSIS IN A PATIENT WITH THALASSEMIA MAJOR DURING TREATMENT WITH THE ORAL IRON CHELATOR, 1,2-DIMETHYL-3-HYDROXYPYRID-4-ONE

Agranulocytosis developed in a 20-year-old Greek patient with beta-tha lassaemia major, 11 weeks after commencing chelation with the oral iro n chelator 1,2-dimethyl-3-hydroxypyrid-4-one (Ll) and 6 weeks after re ceiving the drug at a total daily dose of 105 mg/kg. The patient prese nted with generalised weakness, low-grade fever and sore throat. The t otal white cell count was 2.0 x 10(9)/1 with 0.1 x 10(9)/1 neutrophils . The patient was admitted to hospital and successfully treated with i ntravenous broad-spectrum antibiotics. Neutrophil count recovered 7 we eks later. A number of immunological tests were performed in an attemp t to elucidate the cause of agranulocytosis. These investigations gave inconclusive evidence for the presence of a weak IgM antibody to myel oid cells exposed to Ll in this patient. Further studies are required, however, to evaluate the mechanism in any other patient who develops agranulocytosis in association with Ll therapy.