Induction of remission with intravenous immunoglobulin and cyclophosphamide in steroid-resistant Evans' syndrome associated with dermatomyositis

Evans' syndrome is characterised by the simultaneous or sequential occurren ce of Coombs'-positive haemolytic anaemia (AIHA) and immune thrombocytopeni a without underlying aetiology. It has been found to be associated with col lagen vascular diseases, especially systemic lupus erythematosus (SLE) and scleroderma. However, Evans' syndrome with dermatomyositis is very rare. A 59-year-old woman, who had been taking high-dose prednisolone for a month a nd cyclosporin for 10 days for dermatomyositis, developed purpura on the le ft popliteal fossa. The platelet and haemoglobin levels decreased to 77 000 /mm(3) and 9.8 g/dl, respectively. Antiplatelet antibody was positive. Thro mbocytopenia responded to intravenous immunoglobulin (IVIG) for a short tim e, but further decreased in a week. Her blood film showed features of haemo lytic anaemia. Laboratory findings showed reticulocytosis and a positive di rect Coombs' test. Bone marrow examination showed a mild hyperplasia of ery throid precursors and megakaryocytes. The patient was successfully treated with cyclophosphamide in addition to oral prednisolone. AIHA in connective tissue disease may develop gradually and show a benign clinical course in m ost patients. Therefore, we suggest that patients with dermatomyositis and anaemia should always be checked for haemolysis if there is no other explan ation.