儿童卡斯尔曼病20例临床诊疗分析

Clinical analysis of 20 cases of Castleman disease in children

目的增加对儿童卡斯尔曼病(CD)的临床表现特点、病理特征及预后的了解,并探讨目前儿童CD的治疗方案。方法对2007年7月至2022年12月苏州大学附属儿童医院经过病理诊断的20例CD病人的临床表现、病理特征、治疗方案、预后等进行回顾性分析。结果20例病人中男性为8例,女性为12例,年龄7.0~15.6岁。临床分类:单中心型CD(UCD)14例(70%),多中心型CD(MCD)6例(30%)。病理分类:透明血管型(HV)14例(70%),浆细胞型(PC)5例(25%),混合型1例(5%)。手术完全切除10例(50%)均为UCD,化疗后再手术切除4例(20%),完全化疗6例(30%),方案采用CHOP(环磷酰胺、阿霉素、长春新碱、泼尼松)、TCP(沙利度胺、环磷酰胺、泼尼松)、R-CVP(利妥昔单抗、环磷酰胺、长春新碱)、托珠单抗联合激素及利妥昔单抗联合大剂量激素治疗。随访至2023年4月,19例均生存,1例因肺部间质病变及噬血细胞综合征死亡。结论儿童CD是一种临床少见疾病,以淋巴结肿大为主要表现,UCD较MCD常见。病理为诊断金标准,病理类型中HV型较PC型多见。UCD经手术治疗后疾病缓解率高,MCD多采用药物治疗,随着药物治疗方案的改善,疾病缓解率不断提升。

Objective To increase the understanding of the clinical features,pathological features and prognosis of Castleman disease(CD)in children,and to explore the current treatment of CD in children.Methods The clinical manifestations,pathological features,treatment plan and prognosis of 20 patients with CD diagnosed pathologically in Children's Hospital of Soochow University from July 2007 to December 2022 were retrospectively analyzed.Results Among the 20 patients,8 were males and 12 were females,aged 7.0~15.6 years.Clinical classification:unicentric type(UCD)14 cases(70%),multicentric type(MCD)6 cases(30%).Pathological classification:14 cases(70%)of clear vascular type(HV),5 cases(25%)of plasma cell type(PC),1 case(5%)of mixed type(mixed).UCD was found in 10 patients(50%)who underwent complete surgical resection,and in 4 patients(20%)who underwent re-surgical resection after chemotherapy,and in 6 patients(30%)who underwent complete chemotherapy.CHOP(cyclophosphamide,doxorubicin,vincristine,prednisone),TCP(thalidomide,cyclophosphamide,prednisone),R-CVP(Rituximab,cyclophosphamide,vincristine)and tozil were adopted Mab combined with hormone and rituximab combined with high-dose hormone therapy.Follow-up to April 2023,19 cases survived,1 case died due to pulmonary interstitial disease and hemophagocytic syndrome.Conclusions CD in children is a rare clinical disease,the main manifestation of which is lymph node enlargement,UCD is more common than MCD.Pathology is the gold standard for diagnosis,and HV is more common than PC.The remission rate of UCD after surgical treatment is high,while MCD is mostly treated with drugs.With the improvement of drug treatment program,the remission rate of disease is constantly increasing.