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相關文獻
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題 名 | Idiopathic Myelofibrosis Associated with Renal Extramedullary Hematopoiesis and Nephrotic Syndrome: Case Report=特發性骨髓纖維化合併腎臟髓外造血及腎病症候群:病例報告 |
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作 者 | 劉廷讚; 陳靖博; 陳維仁; 郭景元; 李建德; | 書刊名 | 長庚醫學 |
卷 期 | 23:3 2000.03[民89.03] |
頁 次 | 頁169-174 |
分類號 | 415.74 |
關鍵詞 | 骨髓纖維化; 髓外造血; 腎病症候群; 環間膜增生性腎絲球腎炎; 痛風; Myelofibrosis; Extramedullary hematopoiesis; Nephrotic syndrome; Mesangial proliferative glomerulonephritis; Gout; |
語 文 | 英文(English) |
英文摘要 | Idiopathic myelofibrosis is characterized by bone marrow fibrosis, anemia, leukoerythroblastosis, and extramedullary hematopoiesis in many organs. Renal abnormalities in idiopathic myelofibrosis have been rarely described in the literature and include extramedullary hematopoiesis in the pararenal or retroperitoneal areas resulting in obstructive uropathy and hemtopoietic cell infiltration in tubulointerstitial area and urolithiasis. These lead to azotemia or acute renal failure, which may respond well to radiotherapy and adjuvant chemotherapy. To our knowledge, there has been only one case report of nephrotic syndrome associated with glomerulonephritis in a myelofibrosis patient; however, no effective treatment was described. Herein, we report the case of a patient with idiopathic myelofibrosis who initially presented with hepatomegaly, anemia, and leukoerythroblastosis. A nephrotic syndrome developed 7 years after initial diagnosis. Renal biopsy disclosed the unique pathological finding of simultaneous mesangial proliferative glomerulonephritis, renal extramedullary hematopoiesis, and gouty nephropathy. Despite treatment with busulfan, proteinuria persisted that implied irreversible glomerular injury and a terminal prognosis. We focus on the unusual pathological finding and the association between nephrotic syndrome and idiopathic myelofibrosis. |
本系統中英文摘要資訊取自各篇刊載內容。