30例高危多系统性朗格汉斯细胞组织细胞增生症的临床病理特征并文献复习

Clinicopathological features and literature review of 30 cases of high-risk multi-system Langerhans cell histiocytosis in children

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.