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肝移植受者中伴有血小板减少、全身性水肿、发热、网状纤维变性/肾功能不全及器官肿大(TAFRO)综合征的特发性多中心Castleman病

Idiopathic Multicentric Castleman Disease With Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Insufficiency, and Organomegaly (TAFRO) Syndrome in a Liver Transplant Recipient.

作者信息

Mashal Fares A, Awad Joseph A, Tillman Benjamin F, Mosse Claudio A, Thandassery Ragesh B

机构信息

Division of Gastroenterology and Hepatology, Central Arkansas Veterans Healthcare System, Little Rock, AR.

Division of Gastroenterology and Hepatology, Veterans Affairs Tennessee Valley Healthcare System, Nashville, TN.

出版信息

ACG Case Rep J. 2024 Jul 27;11(8):e01446. doi: 10.14309/crj.0000000000001446. eCollection 2024 Aug.

Abstract

Idiopathic multicentric Castleman disease is a rare lymphoproliferative disorder that can be potentially fatal without timely diagnosis and treatment. We describe the first-ever reported occurrence of idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever, reticulin fibrosis/renal insufficiency, and organomegaly syndrome in a liver transplant recipient. The diagnosis was challenging as the clinical presentation closely mimicked decompensated cirrhosis, and the profound thrombocytopenia limited tissue diagnosis. However, we were able to establish the diagnosis and treat it effectively. This case report signifies that a high index of suspicion and aggressive treatment can be lifesaving in liver transplant recipients presenting with this rare disorder.

摘要

特发性多中心Castleman病是一种罕见的淋巴增殖性疾病,若不及时诊断和治疗可能会危及生命。我们描述了首例肝移植受者出现特发性多中心Castleman病并伴有血小板减少、全身性水肿、发热、网状纤维纤维化/肾功能不全和器官肿大综合征的病例。由于临床表现与失代偿期肝硬化极为相似,且严重的血小板减少限制了组织诊断,因此诊断颇具挑战性。然而,我们成功确诊并进行了有效治疗。本病例报告表明,对于出现这种罕见疾病的肝移植受者,高度的怀疑指数和积极的治疗可挽救生命。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed94/11286246/748a3d964145/ac9-11-e01446-g001.jpg

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