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解析特发性多中心Castleman病的复杂性及其多系统关联:一例报告

Unraveling the Complexities of Idiopathic Multicentric Castleman Disease and Its Multi-systemic Associations: A Case Report.

作者信息

Palvia Aadi R, Saha Prince, Nandi Akshay Rahul, Damera Abhiram Rao, Suresh Aditya

机构信息

Internal Medicine, Kharghar Medicity Hospital, Navi Mumbai, IND.

Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.

出版信息

Cureus. 2024 Jul 19;16(7):e64935. doi: 10.7759/cureus.64935. eCollection 2024 Jul.

Abstract

Castleman disease (CD) comprises a rare spectrum of disorders characterized by benign lymphoepithelial proliferation, classified into unicentric and multicentric forms. The idiopathic multicentric Castleman disease (iMCD) subtype, specifically, is challenging to diagnose and treat due to its variable manifestations and unpredictable disease course. We report a case of a 23-year-old female with a history of iron deficiency anemia presenting with concurrent antiphospholipid syndrome (APS) and human herpesvirus-6 (HHV-6) positivity. Investigations revealed a gastric mass, with a biopsy suggestive of the plasma cell variant of CD. This case report aims to understand the possible association of HHV-6 positivity with CD and the significance of diagnosing APS early in patients with the disease. Treatment with siltuximab and tocilizumab proved effective, highlighting the role of interleukin 6 (IL-6) in the elusive etiology of this condition.

摘要

Castleman病(CD)是一类罕见的疾病谱,其特征为良性淋巴上皮增生,分为单中心型和多中心型。具体而言,特发性多中心Castleman病(iMCD)亚型因其表现多样且病程不可预测,诊断和治疗颇具挑战性。我们报告一例23岁女性病例,该患者有缺铁性贫血病史,同时并发抗磷脂综合征(APS)且人疱疹病毒6型(HHV-6)呈阳性。检查发现胃部有肿物,活检提示为CD的浆细胞变异型。本病例报告旨在了解HHV-6阳性与CD之间可能存在的关联,以及在该病患者中早期诊断APS的意义。使用西妥昔单抗和托珠单抗治疗证明有效,突出了白细胞介素6(IL-6)在这种病因不明疾病中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4eb6/11332968/1fa78cdaa5ea/cureus-0016-00000064935-i01.jpg

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