Scleroderma associated with anemia and thrombocytopenia that responded well to cyclosporin

A 55-year-old woman came to our hospital because of cutaneous sclerosis of the limbs in September 1996, and was diagnosed with scleroderma based on a skin biopsy. In August 1997, the cutaneous sclerosis became progressive (he moglobin level, 4.3 g/dl; platelet count, 7 x 10(9)/l). The laboratory resu lts were positive for the direct Coombs test, bone marrow aspiration showed a dry tap, and the bone marrow biopsy showed marked fatty marrow. Indium-1 11 bone marrow scintigraphy showed a markedly decreasing uptake. These find ings indicated bone marrow hypoplasia associated with hemolytic anemia. Aft er prednisolone therapy (60 mg) was initiated, the direct Coombs test becam e negative but the blood cell count did not increase. Then, 300 mg of cyclo sporin was initiated and anemia and thrombocytopenia improved. The cyclospo rin dosage was gradually decreased and the patient's hematological conditio n was good, although the cutaneous sclerosis changed only a little. This is a rare and interesting case of a patient with scleroderma associated with bone marrow insufficiency and hemolysis who responded well to cyclosporin. Copyright (C) 2001 S. Karger AG, Basel.