Bone marrow necrosis successfully treated with corticosteroid

A 60-yr-old white woman with CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, telangectasia), idiophatic portal hypertension and esophageal varices presented with a 2-month history of bone pain and severe anemia, requiring erythrocyte transfusion every 5-7 d. Initial laboratory findings were hemoglobin (Hb) 4.4 g/dL, platelets 15 x 10(9)/L, white blood cell count (WBC) 2.7 x 10(9)/L. Bone marrow biopsy showed large areas of BMN, and cultures of bone marrow aspirate were negative. The patient was started on intravenous pulse methylprednisolone (1000 mg/d for 3 d) followed by oral prednisone (1 mg/kg/d), and did not require erythrocyte transfusion thereafter. On the 40th day, our patient had Hb 11.6 g/dL, platelets 120 x 10(9)/L, WBC 6.2 x 10(9)/L. Here, we describe the first report of BMN in a patient with CREST syndrome, the first description of successful treatment with intravenous pulse corticosteroid and discuss the possible immune mechanisms involved in the present case.