儿童Castleman病21例临床回顾性分析
收稿日期: 2021-06-25
网络出版日期: 2022-02-11
基金资助
恶性肿瘤化疗后骨髓抑制诊疗规范的推广与应用(cstc2018jscx msybX0102)
A retrospective clinical analysis of 21 cases of Castleman disease in children
Received date: 2021-06-25
Online published: 2022-02-11
目的 分析Castleman病（CD）的临床特点,以期提高临床医师对CD的认识。方法 回顾性分析2010年1月—2020年6月确诊的CD患儿的临床资料,总结归纳患儿的临床特征及诊疗方案、预后。结果 21例CD患儿中,男11例、女10例,中位就诊年龄10.9（5.4~12.1）岁,中位病程为6.0（1.5~16.0）月。其中单中心型CD（UCD）15例、多中心型CD（MCD） 6例。UCD患儿行手术切除,MCD予以化疗、抗IL-6、激素等治疗。中位随访时间37（27~71）月。2例UCD死于合并症,分别为副肿瘤天疱疮（PNP）及肺间质疾病,其余全部存活。结论 CD在儿童中罕见,早期发现,诊疗及时、得当,总体预后良好。无症状UCD患儿首选手术治疗,治愈率高。MCD患儿予以抗IL-6及激素、化疗等治疗,长期存活率明显提高。
关键词： Castleman病; 临床表现; 治疗方式; 预后
刘智超 , 李长春 , 王珊 , 孔祥如 , 章均 , 杨超 , 赵珍珍 . 儿童Castleman病21例临床回顾性分析[J]. 临床儿科杂志, 2022 , 40(2) : 139 -143 . DOI: 10.12372/jcp.2022.21e0963
Objective To analyze clinical features of Castleman disease (CD) for improving clinicians' understanding of CD. Methods Data including clinical manifestation, diagnosis, therapy and prognosis of pediatric patients with CD diagnosed and treated in a single center in Children's Hospital of Chongqing Medical University from January, 2010 to June, 2020 were retrospectively analyzed. Results There are 21 children with CD (11 males and 10 females), the median age of patients was 10.90 (5.35, 12.1) years old. All patients have performed CT examinations. Among them, all unicentric CD (UCD) patients underwent complete surgical resection, and six patients of multicentric CD (MCD) were treated with chemotherapy, anti-IL-6 and hormones. The median follow-up time of 21cases is 37 months (27,71), 2 UCD patients died with paraneoplastic pemphigus and lung interstitial changes. Conclusion Castleman disease is rare in pediatric patients, and the overall prognosis is well if it was treated timely. For UCD patients, preferred surgical treatment for asymptomatic UCD children have high cure rate. Children with MCD treated with anti-IL-6, hormones, and chemotherapy have significantly improved long-term survival rates.
Key words： Castleman disease; clinical manifestation; therapy; prognosis
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