Zhang Xuanye, Rao Huilan, Xu Xiaolu, Li Zhihua, Liao Bing, Wu Hongmei, Li Mei, Tong Xiuzhen, Li Juan, Cai Qingqing
Department of Medical Oncology, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Guangzhou, China.
Department of Pathology, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Guangzhou, China.
Cancer Sci. 2018 Jan;109(1):199-206. doi: 10.1111/cas.13439. Epub 2017 Dec 28.
Castleman disease (CD) is a rare lymphoproliferative disorder. To assess the clinical features, outcomes, and prognostic factors of this disease, we retrospectively analyzed 185 HIV-negative CD patients from four medical centers in southern China. The median age was 37 years. One hundred and twenty-one patients (65.4%) were classified as unicentric CD (UCD) and 64 patients (34.6%) were classified as multicentric CD (MCD). The histology subtype was hyaline-vascular for 132 patients (71.4%), plasma cell for 50 patients (27%), and mixed type for 3 patients (1.6%). The 5-year overall survival (OS) of 185 CD cases was 80.3%. All UCD patients underwent surgical excision, whereas the treatment strategies of MCD patients were heterogeneous. The outcome for UCD patients was better than MCD patients, with 5-year OS rates of 93.6% and 51.2%, respectively. In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (≥40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis.
卡斯特曼病（CD）是一种罕见的淋巴增生性疾病。为评估该疾病的临床特征、预后及预后因素，我们对来自中国南方四个医疗中心的185例HIV阴性的CD患者进行了回顾性分析。中位年龄为37岁。121例患者（65.4%）被归类为单中心型CD（UCD），64例患者（34.6%）被归类为多中心型CD（MCD）。组织学亚型为透明血管型的有132例患者（71.4%），浆细胞型的有50例患者（27%），混合型的有3例患者（1.6%）。185例CD病例的5年总生存率（OS）为80.3%。所有UCD患者均接受了手术切除，而MCD患者的治疗策略则各不相同。UCD患者的预后优于MCD患者，5年OS率分别为93.6%和51.2%。在对MCD亚组的进一步分析中，使用Cox回归模型进行的多因素分析显示，年龄、脾肿大和治疗前血清白蛋白水平是OS的独立预后因素。这项包含迄今为止最大样本量的多中心研究表明，MCD是一种与UCD不同的疾病，预后明显更差。年龄较大（≥40岁）、脾肿大和低白蛋白血症是MCD预后较差的危险因素。
