儿童多系统性朗格汉斯细胞组织细胞增生症8例临床病理分析

Multi-system Langerhans cell histiocytosis in children:a clinicopathologic study of 8 cases

目的:探讨多系统朗格汉斯细胞组织细胞增生症(multi-system Langerhans cell system,MS-LCH)的临床特点、病理诊断、鉴别诊断及预后等。方法:结合相关文献,对8例MS-LCH的临床资料、病理学表现和免疫组织化学结果进行分析。结果:男性1例,女性7例,年龄从13 d-3岁5个月,其中6例累及高危器官。临床表现为发热、皮疹或贫血,伴有肝、脾、淋巴结、骨、垂体等多系统器官受累。镜下见受累器官内朗格汉斯组织细胞增生,核呈分叶状或不规则形。免疫组化显示朗格汉斯细胞阳性表达CD1a和S-100。结论:MS-LCH临床表现复杂,诊断需要结合临床表现、影响学检查及组织病理学检查。累及高危器官者,预后较差。

Objective： To study clinical and histopathological characteristics, diagnostic criteria, differential diagnosis and prognosis of systematic Langerhans cell histiocytosis in children. Methods： Clinical and pathological features were studied in 8 cases of multi-system Langerhans cell histiocytosis with review of the literatures. Results： There were 1 males and 7 females. The youngest was 13-day-old and the oldest was 3 years and 5 months old. Among those MS-LCH patients, 6 patients had risk organ involvement. The clinical symptoms included fever, skin eruption or anemia, with infiltrative lesion of liver, spleen, lymph node, bone and hematopoietic system. Microscopically, the disease was characterized by clonal hyperplasia of histiocytes with folded nucleus. Immunohistochemical staining showed that the histiocytes were positive for CD la and S-100. Conclusion： Multi-system Langerhans cell histiocytosis presents complex dinical symptoms. The diagnosis needs a combination of clinical features, image examination and histopathological characters. If the risk organ was involved, the prognosis is poor.