Castleman病临床诊治分析

目的：分析Castleman病（Castleman disease, CD）的病理及临床特征，探索本中心CD患者临床、病理特点以及与生存的关系。方法：回顾性研究本院95例病理确诊为CD患者的临床病理特点、治疗及生存资料。结果：本中心CD患者病理亚型以透明血管型（HV）较多（56/95）；单中心型（UCD）较多（69/95），21例患者为特发性多中心型Castleman病-非特指型（iMCD-NOS）。UCD及MCD患者间比较，多项基线临床资料及实验室检查有统计学显著性差异。部分患者存在淋巴细胞绝对值降低。总体预测3年PFS为78.8%；预测3年OS为92.2%。MCD患者预测3年OS为78.2%，预测5年OS为70.2%。单因素分析示影响PFS的因素包括病理亚型、病灶数目、基线是否存在B组症状、脾大、浆膜腔积液、球蛋白增高、CRP增高。影响OS的因素包括病理亚型，病灶数目、基线是否存在脾大、浆膜腔积液、CRP增高。结论：本中心CD患者合并脾大，浆膜腔积液等情况的比例相对较低；部分患者合并淋巴细胞绝对值降低。MCD患者预测5年OS优于国内外报道。病理亚型、受累部位、是否存在脾大或浆膜腔积液、CRP等炎症标志物是否升高可能是影响患者PFS及OS的标志物。

Clinical features analysis of Castleman disease patients

Objective To analyze clinical pathological and therapeutic features of Castleman disease (CD). Methods Totally, 95 patients were enrolled and their clinical features, lablatory results, pathological features, treatment and survival data were collected. Results Among the CD patients, hyaline vascular pathological subtype accounted for 58.9% (56/95); Uni-center CD (UCD) accounted for 72.6% (69/95) while muitl-center CD (MCD) accounted for 27.4% (26/95). Twenty-one patients had idiopathic MCD-not otherwise specified (iMCD-NOS) subtype. There were significant differences in the pathological subtype, maximum diameter of lesions, the ratios of B symptoms, splenomegaly, and serous effusion, and several laboratory factors between UCD and MCD subgroup (P<0.05). Laboratory examination showed lymphocytopenia happened in 39(41.1%) patients.UCD and asymptomatic MCD (aMCD) patients were free from systemic medication,while iMCD patients were undertaken systemic medication including glucocorticoid, immunomodulatory drugs, rituximab and/or chemotherapy. For all the patients, estimated 3-year progression-free survival (PFS) and overall survival (OS) rates were 78.8% and 92.2%; While for MCD subgroup, estimated 3-year OS and 5-year OS rates were 78.2% and 70.2%. Conclusions The proportions of splenomegaly and serous effusion are low in CD patients, while the lymphocytes decreases, and estimated 5-year OS rate in MCD patients is better than previously reports.