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五处活检,一个诊断:特发性多中心 Castleman 病的挑战。

Five biopsies, one diagnosis: challenges in idiopathic multicentric Castleman disease.

机构信息

Department of Medicine, University of Toronto, Toronto, Ontario, Canada

Department of Medicine, Division of Nephrology, University of Toronto, Toronto, Ontario, Canada.

出版信息

BMJ Case Rep. 2020 Nov 23;13(11):e236654. doi: 10.1136/bcr-2020-236654.

DOI:10.1136/bcr-2020-236654
PMID:33229481
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7684809/
Abstract

A previously healthy 29-year-old man initially presented to the hospital with pleuritic chest pain and shortness of breath. Over the next 2 months he developed ongoing fevers and night sweats with recurrent exudative pleural effusions and ascites. He had an extensive infectious and autoimmune workup that was unremarkable. He had an initial lymph node biopsy that showed reactive changes only. He had an acute kidney injury and his renal biopsy revealed thrombotic microangiopathy. His liver biopsy showed non-specific inflammatory changes. His bone marrow biopsy showed megakaryocyte hyperplasia and fibrosis, which raised suspicion for the thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction and organomegaly syndrome subtype of multicentric Castleman disease. This prompted a repeat lymph node biopsy, showing changes consistent with mixed type Castleman disease that fit with his clinical picture. He was initiated on steroids and siltuximab with significant clinical improvement.

摘要

一位既往健康的 29 岁男性最初因胸痛和呼吸急促到医院就诊。在接下来的 2 个月中,他持续发热和盗汗,反复出现渗出性胸腔积液和腹水。他进行了广泛的感染和自身免疫检查,但均未见异常。最初的淋巴结活检仅显示反应性改变。他出现急性肾损伤,肾脏活检显示血栓性微血管病。肝脏活检显示非特异性炎症改变。骨髓活检显示巨核细胞增生和纤维化,这提示他患有特发性血小板减少性紫癜、腹水、网状纤维纤维化、肾功能不全和器官肿大综合征型多发性骨髓瘤。这促使他再次进行淋巴结活检,结果显示符合他临床表现的混合型 Castleman 病改变。他开始接受类固醇和西妥昔单抗治疗,临床症状显著改善。

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引用本文的文献

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Atypical clinical presentation associated with Castleman disease: a case report and review of the literature.与Castleman病相关的非典型临床表现:一例病例报告及文献复习
Front Med (Lausanne). 2025 Aug 21;12:1626722. doi: 10.3389/fmed.2025.1626722. eCollection 2025.

本文引用的文献

1
Overview of Castleman disease.卡斯特曼病概述。
Blood. 2020 Apr 16;135(16):1353-1364. doi: 10.1182/blood.2019000931.
2
Case report on mesangial proliferative glomerulonephritis with multicentric Castleman's disease: Approach to the onset mechanism of immunoglobulin A nephropathy.特发性系膜增生性肾小球肾炎合并多中心型 Castleman 病 1 例报告:IgA 肾病发病机制探讨。
Clin Immunol. 2020 Mar;212:108347. doi: 10.1016/j.clim.2020.108347. Epub 2020 Jan 21.
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HHV-8-negative multicentric Castleman disease presenting as a crescentic immune complexes membranoproliferative glomerulonephritis.HHV-8阴性多中心性Castleman病表现为新月形免疫复合物膜增生性肾小球肾炎。
BMJ Case Rep. 2020 Jan 6;13(1):e231844. doi: 10.1136/bcr-2019-231844.
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Thrombotic microangiopathy on kidney biopsy in a patient with TAFRO syndrome.TAFRO综合征患者肾活检显示血栓性微血管病。
CEN Case Rep. 2018 Nov;7(2):243-247. doi: 10.1007/s13730-018-0338-x. Epub 2018 May 15.
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TAFRO Syndrome in Caucasians: A Case Report and Review of the Literature.高加索人群中的TAFRO综合征:一例报告及文献综述
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International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.HHV-8阴性/特发性多中心Castleman病的国际循证共识诊断标准。
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