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Obstructive Uropathy as an Initial Presentation of Primary Myelofibrosis: Case Report and Review of Literature

机译:梗阻性尿病作为原发性骨髓纤维化的初步表现:病例报告和文献复习

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摘要

Primary myelofibrosis (PMF) is a rare hematological disorder associated with progressive cytopenia and extra-medullary hematopoiesis. Acute kidney injury in this disease has been reported from diverse etiologies such as renal and peri-renal extramedullary hematopoiesis and tumor lysis syndrome. We report a patient who presented with obstructive uropathy from uric acid stones who was incidentally diagnosed with PMF during workup for persistent thrombocytosis and leukocytosis. Marrow histopathology was unique in presenting features of early PMF despite clinical picture mimicking essential thrombocythemia. Despite a common background of hyperuricemia in myeloproliferative neoplasms, AKI resulting from urate nephrolithiasis has seldom been reported in PMF. Published data on this association and clinical management is reviewed briefly.
机译:原发性骨髓纤维化(PMF)是一种与进行性血细胞减少和髓外造血相关的罕见血液病。据报道,该病的急性肾脏损伤来自多种病因,例如肾和肾周围髓外造血和肿瘤溶解综合征。我们报道了一名患者因尿酸结石而出现阻塞性尿道病,该患者在持续性血小板增多症和白细胞增多症的检查期间被偶然诊断为PMF。尽管临床表现模仿了原发性血小板增多症,但骨髓组织病理学在呈现早期PMF的特征方面是独特的。尽管在骨髓增生性肿瘤中普遍存在高尿酸血症的背景,但在PMF中很少报道由尿酸肾结石病引起的AKI。简要回顾了有关该协会和临床管理的已发表数据。

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