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肾炎性肌纤维母细胞瘤合并血友病 A 携带者:病例报告及文献复习。

Renal inflammatory myofibroblastic tumor coexisting with hemophilia A carrier: a case report and literature review.

机构信息

Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No.1 Shuaifuyuan Wangfujing, Dongcheng, Beijing, 100730, China.

Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China.

出版信息

CEN Case Rep. 2024 Jun;13(3):199-203. doi: 10.1007/s13730-023-00828-0. Epub 2023 Oct 28.

Abstract

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.

摘要

肾脏炎性肌纤维母细胞瘤(IMT)是一种非常罕见的疾病。本报告介绍了首例伴甲型血友病携带者状态的肾脏 IMT 的病例。一名 52 岁无症状女性在一次为期 5 个月的常规计算机断层扫描(CT)扫描中偶然发现肾脏肿块。超声和增强 CT 扫描提示可能为肾脏恶性肿瘤。20 多年前,该患者在分娩时经历了严重出血,且患有类风湿性关节炎 5 年。术后期间,给予静脉补充第八因子。在用力排便后,患者出现血尿。持续的凝血因子补充缓解了血尿症状。IMT 的根本原因和发病机制尚不清楚。IMT 常与类风湿关节炎相关,可能与其病因有关。手术切除是主要的治疗方法,复发极为罕见。在伴有血友病的情况下,需要密切监测凝血功能并适当补充凝血因子。

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本文引用的文献

1
Uterine inflammatory myofibroblastic tumor.子宫炎性肌纤维母细胞瘤。
Pathol Res Pract. 2023 Feb;242:154335. doi: 10.1016/j.prp.2023.154335. Epub 2023 Jan 23.
8
Consensus on hemophilia in Mexico.墨西哥血友病共识。
Gac Med Mex. 2021;157(Suppl 1):S1-S35. doi: 10.24875/GMM.M21000463.
9
Renal inflammatory myofibroblastic tumor: A case report.肾炎性肌纤维母细胞瘤:一例报告。
Urol Case Rep. 2021 Mar 3;37:101620. doi: 10.1016/j.eucr.2021.101620. eCollection 2021 Jul.
10
Genetic causes of haemophilia in women and girls.女性和女童血友病的遗传学病因。
Haemophilia. 2021 Mar;27(2):e164-e179. doi: 10.1111/hae.14186. Epub 2020 Dec 13.

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