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Keywords:

  • bone marrow necrosis;
  • cytopenia;
  • corticosteroid;
  • CREST syndrome

Abstract:  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 × 109/L, white blood cell count (WBC) 2.7 × 109/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 × 109/L, WBC 6.2 × 109/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.