Clinical Characteristics and Outcomes of Castleman Disease: A Multicenter Consortium Study of 428 Patients With 15-Year Follow-Up

Authors

Wanying Liu
Qingqing Cai
Tiantian Yu
Paolo Strati
Frederick B Hagemeister
Qiongli Zhai
Mingzhi Zhang
Ling Li
Xiaosheng Fang
Jianyong Li
Ruifang Sun
Shanxiang Zhang
Hanjin Yang
Zhaoming Wang
Wenbian Qian
Noriko Iwaki
Yasuharu Sato
Eric Oksenhendler
Zijun Y Xu-Monette
Ken H Young
Li Yu

Publication Date

1-1-2022

Journal

American Journal of Cancer Research

Abstract

Castleman disease (CD) has been reported as a group of poorly understood lymphoproliferative disorders, including unicentric CD (UCD) and idiopathic multicentric CD (iMCD) which are human immunodeficiency virus (HIV) negative and human herpes virus 8 (HHV-8) negative. The clinical and independent prognostic factors of CD remain poorly elucidated. We retrospectively collected the clinical information of 428 patients with HIV and HHV-8 negative CD from 12 large medical centers with 15-year follow-up. We analyzed the clinicopathologic features of 428 patients (248 with UCD and 180 with iMCD) with a median age of 41 years. The histology subtypes were hyaline-vascular (HV) histopathology for 215 patients (56.58%) and plasmacytic (PC) histopathology for 165 patients (43.42%). Most patients with UCD underwent surgical excision, whereas the treatment strategies of patients with iMCD were heterogeneous. The outcome for patients with UCD was better than that for patients with iMCD, 5-year overall survival (OS) rates were 95% and 74%, respectively. In further analysis, a multivariate analysis using a Cox regression model revealed that PC subtype, hepatomegaly and/or splenomegaly, hemoglobin ≤ 80 g/L, and albumin ≤ 30 g/L were independent prognostic factors of CD for OS. The model of iMCD revealed that age > 60 years, hepatomegaly and/or splenomegaly, and hemoglobin ≤ 80 g/L were independent risk factors. In UCD, single-factor analysis identified two significant risk factors: hemoglobin ≤ 100 g/L and albumin ≤ 30 g/L. Our study emphasizes the distinction of clinical characteristics between UCD and iMCD. The importance of poor risk factors of different clinical classifications may direct more precise and appropriate treatment strategies.

Keywords

Castleman disease, clinical characteristics, risk prognostic factors, treatment

PMID

36225639

PMCID

PMC9548017

PubMedCentral® Posted Date

9-15-2022

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes