Liu Wanying, Cai Qingqing, Yu Tiantian, Strati Paolo, Hagemeister Frederick B, Zhai Qiongli, Zhang Mingzhi, Li Ling, Fang Xiaosheng, Li Jianyong, Sun Ruifang, Zhang Shanxiang, Yang Hanjin, Wang Zhaoming, Qian Wenbian, Iwaki Noriko, Sato Yasuharu, Oksenhendler Eric, Xu-Monette Zijun Y, Young Ken H, Yu Li
Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University NanChang, China.
State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Sun Yat-sen University Cancer Center Guangzhou, China.
Am J Cancer Res. 2022 Sep 15;12(9):4227-4240. eCollection 2022.
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.
卡斯特曼病（CD）被报道为一组了解甚少的淋巴增殖性疾病，包括单中心性CD（UCD）和特发性多中心性CD（iMCD），它们均为人类免疫缺陷病毒（HIV）阴性和人类疱疹病毒8型（HHV-8）阴性。CD的临床及独立预后因素仍未得到充分阐明。我们回顾性收集了来自12家大型医疗中心的428例HIV和HHV-8阴性CD患者的临床信息，并进行了15年的随访。我们分析了428例患者（248例UCD和180例iMCD）的临床病理特征，患者的中位年龄为41岁。组织学亚型中，215例患者（56.58%）为透明血管（HV）组织病理学类型，165例患者（43.42%）为浆细胞（PC）组织病理学类型。大多数UCD患者接受了手术切除，而iMCD患者的治疗策略则各不相同。UCD患者的预后优于iMCD患者，5年总生存率（OS）分别为95%和74%。在进一步分析中，使用Cox回归模型进行的多因素分析显示，PC亚型、肝肿大和/或脾肿大、血红蛋白≤80 g/L以及白蛋白≤30 g/L是CD患者OS的独立预后因素。iMCD模型显示，年龄>60岁、肝肿大和/或脾肿大以及血红蛋白≤80 g/L是独立危险因素。在UCD中，单因素分析确定了两个显著危险因素：血红蛋白≤100 g/L和白蛋白≤30 g/L。我们的研究强调了UCD和iMCD临床特征的差异。不同临床分类中不良危险因素的重要性可能指导更精确和恰当的治疗策略。
