title: Bone marrow necrosis successfully treated with corticosteroid.
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 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.
authors.affiliation: Pulmonary Division, University of São Paulo Medical School, São Paulo, Brazil. alfredonicodemos@hotmail.com
authors.forename: Alfredo N C
authors.initials: AN
authors.lastname: Santana
authors.forename: Renata G
authors.initials: RG
authors.lastname: Ramos
authors.forename: Eduardo F
authors.initials: EF
authors.lastname: Zanandrea
authors.forename: Rodrigo A
authors.initials: RA
authors.lastname: Brandão-Neto
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001853:Bone Marrow / Q000187:drug effects* / Q000473:pathology*; D017675:CREST Syndrome / Q000150:complications*; D004932:Esophageal and Gastric Varices / Q000150:complications; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009336:Necrosis; D011241:Prednisone / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D011241:Prednisone; D008775:Methylprednisolone