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“这不是狼疮”。胎盘部位滋养细胞肿瘤表现为狼疮样副肿瘤综合征。一个大查房病例。

"It's not lupus". A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case.

机构信息

Amsterdam Rheumatology and Immunology Center (ARC), Department of Rheumatology and Clinical Immunology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Department of Nephrology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

出版信息

Lupus. 2021 Mar;30(3):495-501. doi: 10.1177/0961203320981765. Epub 2021 Jan 6.

DOI:10.1177/0961203320981765
PMID:33407043
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7930597/
Abstract

Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.

摘要

胎盘部位滋养细胞肿瘤(PSTT)是一种罕见的妊娠滋养细胞疾病亚型。PSTT 与肾病综合征的关联极为罕见,迄今为止仅描述了 8 例。所有病例均在症状出现后数月内进行了子宫切除术,导致肾病综合征立即缓解,但有 1 例患者因 PSTT 并发症死亡。我们描述了一位女性的病史,她在肾病综合征发病多年后才发现 PSTT。肾脏活检显示狼疮样系膜毛细血管性肾炎,随着时间的推移,患者出现了其他类似系统性红斑狼疮(SLE)的症状。我们对该临床实体的文献进行了综述,并详细阐述了其病理生理学。此外,我们还反思了锚定偏差的现象,这种现象导致医生在没有根据未解释的肿瘤标志物升高的情况质疑这一诊断的情况下,假设患者患有 SLE。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8262/7930597/a8b222aadc8d/10.1177_0961203320981765-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8262/7930597/94f17de67981/10.1177_0961203320981765-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8262/7930597/a8b222aadc8d/10.1177_0961203320981765-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8262/7930597/94f17de67981/10.1177_0961203320981765-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8262/7930597/a8b222aadc8d/10.1177_0961203320981765-fig2.jpg

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CARE-Placental site trophoblastic tumor presenting with thrombotic microangiopathy: A case report.伴有血栓性微血管病的胎盘部位滋养细胞肿瘤:一例报告
Medicine (Baltimore). 2018 Oct;97(43):e12698. doi: 10.1097/MD.0000000000012698.
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Disappearance of a thrombotic microangiopathy-like glomerular lesion in a patient with a placental site trophoblastic tumor after hysterectomy.
子宫切除术后胎盘部位滋养细胞肿瘤患者血栓性微血管病样肾小球病变消失
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A case of placental site trophoblastic tumor complicating nephrotic syndrome in which hysteroscopic biopsy did not yield a definitive diagnosis.一例胎盘部位滋养细胞肿瘤合并肾病综合征,其中宫腔镜活检未得出明确诊断。
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Historical, morphological and clinical overview of placental site trophoblastic tumors: from bench to bedside.胎盘部位滋养细胞肿瘤的历史、形态学及临床概述:从实验室到临床
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