摘要
目的了解系统性幼年黄色肉芽肿(JXG)的临床特点、治疗及预后情况。方法对4例JXG患儿的临床资料进行回顾性总结并复习相关文献。结果4例患儿均依据临床表现、病理检查及免疫组化染色结果确诊,3例2017年以后就诊的患儿检测了其病理组织和血浆中的BRAF V600E突变,结果均为阴性。男2例,女2例,年龄分别为2岁6月、1岁5月、1岁4月、1岁6月。患儿因皮疹、多饮多尿、骨骼包块就诊。4例患儿累及皮肤、中枢神经系统、骨、肺、甲状腺。4例患儿接受朗格罕细胞组织细胞增生症(LCH)的一线方案化疗,定期评估均达到部分缓解。1例失访,余3例已停药,分别随访了26、22、12个月。复习国内外文献报道。结论系统性JXG常发生在婴幼儿时期,临床表现多样,以LCH方案为基础的化疗,预后良好。
Objective To investigate the clinical characteristics,treatment and prognosis of systemic juvenile xanthogranuloma(JXG).Methods The clinical data of 4 children with systemic JXG were analyzed retrospectively and related literatures were reviewed.Results All 4 cases were diagnosed with systemic JXG according to the clinical features,pathologic examination and immunohistochemical staining.The BRAF V600E mutation in pathological specimens and plasma was detected in 3 cases who were admitted after 2017 and the results were all negative.There were 2 males and 2 females,their ages were 2 years 6 months,1 year 5 months,1 year 4 months and 1 year 6 months respectively.These patients visited the hospital with the chief complaint of rash,bone lumps,polydipsia and polyuria.Their sites of disease were skin,central nervous system,lung,bone,thyroid.These cases were treated with first-line chemotherapy of Langerhans cell histiocytosis(LCH)and all of them achieved partial remission.Except 1 case lost follow-up,other 3 cases have finished the treatment.These 3 cases were followed up for 26,22,and 12 months,respectively.Conclusions Systemic JXG often occurs in infants and toddlers,with various clinical manifestations.Chemotherapy based on LCH regimen make good prognosis and few deaths.
作者
廉红云
李志刚
崔蕾
魏昂
杨颖
程华
何乐健
张莉
赵晓曦
王天有
张蕊
LIAN Hongyun;LI Zhigang;CUI Lei;WEI Ang;YANG Ying;CHENG Hua;HE Lejian;ZHANG Li;ZHAO Xiaoxi;WANG Tianyou;ZHANG Rui(Beijing Key Laboratory of Pediatric Hematology Oncology,National Key Discipline of Pediatrics(Capital Medical University),Key Laboratory of Major Diseases in Children,Ministry of Education,Radiology Department,Pathology department,Hematology Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Laboratory of Hematology and Oncology,Beijing Institute of Pediatrics,Beijing 100045,China)
出处
《中国小儿血液与肿瘤杂志》
CAS
2021年第2期84-88,共5页
Journal of China Pediatric Blood and Cancer
关键词
幼年黄色肉芽肿
系统性
LCH治疗方案
Juvenile Xanthogranuloma
Systemic
Histiocyte Society′s Langerhans cell histiocytosis protocol
