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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 analyze the clinical and histopathological features of multiple systemic Langerhans cell histiocytosis and to explore its pathogenesis, diagnosis, differential diagnosis and prognosis. Methods The clinical data, histopathological findings and immunohistochemical results of 5 cases of children with Langerhans cell histiocytosis were analyzed and the literature review was reviewed. Results There were 2 males and 3 females. The youngest was 49 days and the maximum was 27 months. Most patients were admitted to hospital with fever, anemia or rash and were involved with multiple organs such as liver, spleen, lymph nodes, bone and lung. Microscopic examination: affected organs within the tissue-like cells hyperplasia, the nucleus was oval, kidney-shaped or irregular shape, showing obvious nuclear ditch and nuclear folding, chromatin delicate, thin nuclear, with varying amounts of eosinophil infiltration . Immunohistochemistry: Tissue cells CD1a and S-100 (+), CD68 and lysozyme were scattered (+), LCA, CK, CD20, CD79a, CD3 and ALK (-). Conclusions The clinical manifestations of multisystem Langerhans cell histiocytosis are complicated. The diagnosis should be combined with clinical manifestations, imaging examination and histopathology examination. The patient’s age of onset is smaller, the more organs involved, the more impaired organ function, the worse the prognosis.