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卡斯特曼病的临床特征:一项系统评价和荟萃分析。

The clinical picture of Castleman disease: a systematic review and meta-analysis.

作者信息

Hoffmann Christian, Oksenhendler Eric, Littler Sarah, Grant Lisa, Kanhai Karan, Fajgenbaum David C

机构信息

Infektionsmedizinisches Centrum Hamburg Study Center, Hamburg, Germany.

Department of Medicine, University of Schleswig-Holstein, Campus Kiel, Kiel, Germany.

出版信息

Blood Adv. 2024 Sep 24;8(18):4924-4935. doi: 10.1182/bloodadvances.2024013548.

Abstract

Castleman disease (CD) encompasses a spectrum of rare disorders, including unicentric CD (UCD), idiopathic multicentric CD (iMCD), and human herpesvirus 8-associated MCD (HHV8+ MCD). We performed a systematic review of publications reporting ≥5 cases of CD between 1995 and 2021, following preferred reporting items for systematic reviews and meta-analyses guidelines, to describe and compare subtypes. We extracted data on clinical symptoms and laboratory parameters as stated in international consensus diagnostic criteria for iMCD and estimated the frequency of each criterion using meta-analyses. We analyzed 32 studies describing 559 UCD, 1023 iMCD, and 416 HHV8+ MCD cases. Although many symptoms and laboratory abnormalities occurred at similar rates in patients with iMCD and HHV8+ MCD, patients with HHV8+ MCD had significantly higher rates of constitutional symptoms (46.6% vs 98.6%; P = .038) and splenomegaly (48.2% vs 89.2%; P = .031). Renal dysfunction was significantly more common in patients with iMCD than in patients with HHV8+ MCD before adjustment (36.9% vs 17.4%; P = .04; adjusted P = .1). Patients with UCD had lower rates of symptoms and laboratory abnormalities, although these were present in 20% of patients and were particularly pronounced in pediatric UCD. There are many similarities in the symptomatology of iMCD and HHV8+ MCD; many patients experience constitutional symptoms and organ dysfunction. Differences between these subtypes likely reflect differences in pathophysiology and/or comorbidity burdens.

摘要

Castleman病(CD)涵盖一系列罕见疾病,包括单中心Castleman病(UCD)、特发性多中心Castleman病(iMCD)和人类疱疹病毒8相关的Castleman病(HHV8+MCD)。我们按照系统评价和Meta分析的首选报告项目指南,对1995年至2021年间报告≥5例Castleman病的出版物进行了系统评价,以描述和比较各亚型。我们提取了国际iMCD共识诊断标准中所述的临床症状和实验室参数数据,并使用Meta分析估计了每个标准的频率。我们分析了32项研究,这些研究描述了559例UCD、1023例iMCD和416例HHV8+MCD病例。尽管iMCD和HHV8+MCD患者出现许多症状和实验室异常的发生率相似,但HHV8+MCD患者出现全身症状(46.6%对98.6%;P = 0.038)和脾肿大(48.2%对89.2%;P = 0.031)的发生率显著更高。在调整前,iMCD患者肾功能不全的发生率显著高于HHV8+MCD患者(36.9%对17.4%;P = 0.04;调整后P = 0.1)。UCD患者出现症状和实验室异常的发生率较低,尽管20%的患者存在这些情况,且在儿童UCD中尤为明显。iMCD和HHV8+MCD的症状学有许多相似之处;许多患者有全身症状和器官功能障碍。这些亚型之间的差异可能反映了病理生理学和/或合并症负担的差异。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6f6/11421328/5c7cc4e76a6d/BLOODA_ADV-2024-013548-ga1.jpg

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