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目的通过一例朗格罕氏细胞肉瘤病例特点的总结及相关文献复习,提高对本病的认识。方法临床病例分析及文献复习。结果临床症状均以发热、全身淋巴结肿大、肝脾肿大为特点,病理以细胞呈圆形或椭圆形,大小不一,异形明显,胞浆丰富,颗粒增多为特征,免疫分型以CD1a+,S-100蛋白+为表现,治疗以化疗为主。结论朗格罕氏细胞肉瘤是一种罕见的树突状细胞恶性肿瘤,化疗效果不佳,预后差。
Objective To summarize the characteristics of a case of Langerhans cell sarcoma and review the relevant literature to improve the understanding of the disease. Methods Clinical case analysis and literature review. Results The clinical symptoms were characterized by fever, generalized lymph nodes and hepatosplenomegaly. The pathological features were round or oval cells with different sizes, distinct shaped cells, abundant cytoplasm and increased granules. The immunophenotypes included CD1a + , S-100 protein + performance, the treatment of chemotherapy-based. Conclusion Langerhans cell sarcoma is a rare dendritic cell malignant tumor with poor efficacy and poor prognosis.