儿童多系统性朗格汉斯细胞组织细胞增生症5例临床病理分析

Clinicopathologic analysis of systematic Langerhans cell histiocytosis in children

目的分析多系统性朗格汉斯细胞组织细胞增生症的临床和组织病理学特点,探讨其发病机制、诊断、鉴别诊断及预后特点。方法对5例儿童多系统性朗格汉斯细胞组织细胞增生症的临床资料、组织病理学表现和免疫组化结果进行分析,并结合文献复习。结果男性2例,女性3例,年龄最小49天,最大27个月;多以发热、贫血或皮疹入院,伴肝、脾、淋巴结、骨、肺等多系统器官受累。镜检:受累器官内组织细胞样细胞增生,核呈椭圆形、肾形或不规则形,可见明显核沟和核折叠,染色质细腻,核膜薄,伴数量不等的嗜酸性粒细胞浸润。免疫组化:组织细胞CD1a和S-100(+),CD68和lysozyme散在(+),LCA、CK、CD20、CD79a、CD3和ALK(-)。结论多系统性朗格汉斯细胞组织细胞增生症临床表现复杂,诊断需要结合临床表现、影像学检查及组织病理学检查。该病患者发病年龄越小、受累器官越多、器官功能受损越严重,预后越差。

Objective To study clinical and histopathological characteristics, diagnostic criteria and differential diagnosis of systematic Langerhans cell histiocytosis in children. Methods Clinical and pathological features were studied in 5 cases of systematic Langerhans cell histiocytosis with review of the literatures. Results There were 2 males and 3 females. The youngest was 49 -day -old and the oldest was 27 months. The clinical symptoms included fever, anemia and skin eruption, with infiltrative lesion of liver, spleen, lymph node, bone and lung. Histologically, the disease was characterized by clonal hyperplasia of histiocytes with folded nucleus. Immunohistochemical staining showed that the histiocytes were positive for CDla and S-100, but negative for LCA, CK, CD20, CD79a, CD3 and ALK. Conclusions Systematic Langerhans cell histiocytosis presents complex clinical symptoms. The diagnosis needs a combination of clinical features, image examination and histopathological characters. Differential diagnosis of the disease includes osteomyelitis, juvenile xanthogranuloma, anaplastic large celt lymphoma, sinus histiocytosis with massive lymphadenopathy and Langerhans cell sarcoma. The prognosis of Letterer-Siwe disease is poor, depending on the age, the number of infiltrated organs and the function of infiltrated organs.