肾炎性肌成纤维细胞肿瘤与血友病A携带者共存:病例报告和文献复习。

IF 1 Q4 UROLOGY & NEPHROLOGY
CEN Case Reports Pub Date : 2024-06-01 Epub Date: 2023-10-28 DOI:10.1007/s13730-023-00828-0
Zhenyu Zhang, Hai Wang, Ying Jiang, Zhongming Huang
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引用次数: 0

摘要

肾脏炎性肌纤维母细胞瘤(IMT)是一种异常罕见的疾病。本报告介绍了第一例肾IMT与血友病A携带者共存的病例。一名52岁的无症状女性在为期5个月的常规计算机断层扫描中偶然发现肾脏肿块。超声检查和增强CT扫描引起了对潜在肾脏恶性肿瘤的怀疑。20多年前,患者在分娩时出现严重出血,她有5年类风湿性关节炎病史。在彻底的外科手术后,在围手术期静脉补充因子VIII。剧烈排便后,患者出现血尿。持续补充凝血因子可缓解血尿症状。IMT的根本原因和发病机制尚不清楚。IMT通常与类风湿性关节炎有关,可能与其病因有关。手术切除是主要的治疗方法,复发是极为罕见的事件。在血友病是一个复杂因素的情况下,必须警惕地监测凝血功能并补充适当的凝血因子。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Renal inflammatory myofibroblastic tumor coexisting with hemophilia A carrier: a case report and literature review.

Renal inflammatory myofibroblastic tumor (IMT) is an exceptionally uncommon occurrence. This report presented the first documented case of renal IMT coexisting with hemophilia A carrier status. A 52-year-old asymptomatic female was incidentally discovered to have a kidney mass during a routine computed tomography (CT) scan spanning a 5-month period. Ultrasonography and contrast-enhanced CT scan raised suspicion of a potential renal malignant tumor. Over 2 decades ago, the patient experienced significant bleeding during childbirth, and she possessed a 5-year history of rheumatoid arthritis. Following a radical surgical procedure, intravenous supplementation of factor VIII was administered during the perioperative period. Subsequent to strenuous defecation, the patient encountered hematuria. Continued coagulation factor supplementation led to alleviation of hematuria symptoms. The underlying causes and pathogenesis responsible for IMT remain unclear. IMT is often associated with rheumatoid arthritis, possibly suggesting a connection to its etiology. Surgical excision stands as the primary approach to treatment, with recurrence being an exceedingly rare event. In instances where hemophilia is a complicating factor, vigilant monitoring of coagulation function and appropriate coagulation factor supplementation is imperative.

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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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