儿童Castleman病临床病理和治疗分析  

Clinicopathological and therapeutic analysis of Castleman′s disease in children

作　　者：孟庆磊[1] 李白[2] 王一铭 刘玉峰[2] 鲍俊涛[1] 王晓晖[1] 张书峰[1] Meng Qinglei;Li Bai;Wang Yiming;Liu Yufeng;Bao Juntao;Wang Xiaohui;Zhang Shufeng(Department of Pediatric Surgery,Henan Provincial People′s Hospital,Zhengzhou 450003,China;Department of Pediatric Hematology and Oncology,Children′s Hospital,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]河南省人民医院小儿外科,郑州450003 [2]郑州大学第一附属医院儿童医院血液肿瘤科,郑州450052

出　　处：《中华儿科杂志》2022年第3期227-231,共5页Chinese Journal of Pediatrics

基　　金：河南省医学科技攻关省部共建重点项目(SBGJ202002114)。

摘　　要：目的探讨儿童Castleman病的临床特点、病理表型、治疗和预后。方法回顾性分析2010年5月至2019年10月在河南省人民医院和郑州大学第一附属医院确诊为Castleman病的15例患儿的临床特点、实验室检查及组织病理学资料。结果15例Castleman病患儿中男12例、女3例,就诊年龄12(10,15)岁,发现肿块至病理确诊的时间为9.0(2.0,13.0)个月,大多数患儿为单中心型(13例)。组织病理学类型多为透明血管型(11例)。单中心型病变最常见于颈部(11例),13例单中心型Castleman病患儿均接受了病灶完整手术切除治疗,随访时间20.0(13.5,50.5)个月,预后良好。多中心型Castleman病患儿2例,病理类型均为混合型,符合特发性Castleman病标准,2例患儿均进行手术部分切除,1例采用利妥昔单抗和泼尼松联合治疗,1例采用沙利度胺和泼尼松联合治疗,随访时间分别为32个月和10个月,均预后良好。结论儿童Castleman病大多数延迟确诊,单中心型为主,最常见的病理类型透明血管型,单中心型多表现为无痛性淋巴结肿大,多中心型常有全身症状,整体预后良好。Objective To investigate the clinical features,pathological phenotype,treatment and prognosis of Castleman′s disease in children.Methods Clinical data of 15 children diagnosed with Castleman′s disease in Henan Provincial People′s Hospital and the First Affiliated Hospital of Zhengzhou University from May 2010 to October 2019 were analyzed retrospectively.The clinical characteristics,laboratory examination and histopathological data were analyzed.Results Among the 15 Castleman's disease patients,12 were males and 3 females.The age of first visit was 12(10,15)years.The time from mass discovery to pathologic diagnosis was 9.0(2.0,13.0)months.The majority of patients were unicentric(13 cases),and the histopathological type was hyaline vascular(11 cases).Unicentric lesions were most common in the neck(11 cases),all 13 patients received complete surgical resection of the lesions,the follow-up time was 20.0(13.5,50.5)months,and the prognosis was good.Two cases were multicentric type,the pathological types were mixed variant,meeting the criteria of idiopathic Castleman′s disease,the two children underwent partial surgical resection,one was treated with rituximab and prednisone and the other was treated with thalidomide and prednisone.The follow-up time was 32 months and 10 month,both of them had good prognosis.Conclusions Most cases of Castleman′s disease in children are diagnosed late,and the unicentric type is dominant.The most common pathological type is hyaline vascular,which is characterized by painless lymphadenopathy,while multicentric type has systemic symptoms and both of them have a good overall prognosis.

关 键 词：巨淋巴结增生 儿童 病理学 临床 预后 

分 类 号：R725.5[医药卫生—儿科]