摘要
目的:探讨高危型多系统朗格汉斯细胞组织细胞增生症(multi-system Langerhans cell histiocytosis,MS-LCH)的临床特点、病理表现及其对LCH-III方案的治疗反应。方法:分析郑州儿童医院30例高危型MS-LCH患儿的临床资料、病理学表现和化疗反应,所有接受治疗患儿采用LCH-III化疗方案。结果:30名患儿中,男17名(56.7%),女13名(43.3%),年龄1 d~12岁(中位1岁3个月),临床表现有发热、皮疹、贫血、肝脾肿大、淋巴结肿大、骨质破坏等,免疫组织化学检查提示阳性表达CD1α+,S-100+,LCH-III方案化疗结束后有效率(完全缓解+好转)为92.6%(25/27)。结论:MSLCH临床表现复杂,诊断须依赖组织病理学检查,LCH-III化疗方案有效率较高。
Objective: To study the clinical and histopathological characteristics, diagnostic criteria and therapeutic effect to the LCH-III protocol of high-risk multi-systematic Langerhans cell histiocytosis in children. Methods: Clinical and pathological features were studied in 30 cases of high-risk multi-system Langerhans cell histiocytosis with review of the literatures. All treated patients were treated on the LCH-III protocol. Results: There were 17 males(56.7%) and 13 females(43.3%). The youngest was 1 day and the oldest was 12 years,the median age was 1 year and 3 months. The clinical symptoms included fever, skin eruption or anemia, w ith infiltrative lesion of liver, spleen, lymph node, bone and hematopoietic system. Immunohistochemical staining showed that the histiocytes were positive for CD1α and S-100. The effective rate was 92.6% when the LCH-III protocol was finished. Conclusion: Multi-system Langerhans cell histiocytosis presents complex clinical symptoms. The diagnosis should be confirmed by histopathological examination of biopsy. The LCH-III protocol could obtain great effective rate.
作者
刘玉洁
刘炜
陶箐
张红梅
赵丽颖
何楠
李慧霞
LIU Yujie;LIU Wei;TAO Jing;ZHANG Hongmei;ZHAO Liying;HE Nan;LI Huixia(Department of Hematology and Oncology;Department of Pathology,Children's Hospital of Henan Province,Children's Hospital Affiliated to Zhengzhou University,Zhengzhou 450000,China)
出处
《临床与病理杂志》
2018年第10期2094-2101,共8页
Journal of Clinical and Pathological Research