淋巴结Langerhans细胞组织细胞增生症1例并文献复习

Langerhans Cell Histiocytosis in Lymph Nodes: A Case Report and Review of the Literature

摘要: Langerhans细胞组织细胞增生症(Langerhans cell histiocytosis, LCH)是一种主要由Langerhans细胞克隆性肿瘤性增生形成的肿瘤性疾病，单系统发生在淋巴结者少见，临床上经常发生误诊。本例患者男性，45岁，以发热伴右腹股沟肿块1个月余入院。光镜下淋巴结基本结构几乎完全被破坏，病变主要由Langerhans细胞和嗜酸性细胞或嗜酸性脓肿组成。Langerhans细胞中等至偏大，胞质丰富嗜酸性，细胞核圆形、卵圆形，咖啡豆样核或不规则，有明显的核沟纹。病灶中有散在的多核巨细胞和数量不等的嗜酸性粒细胞浸润，可见嗜酸性脓肿。免疫组化标记显示瘤细胞表达S-100、CD1a、CD68、vimentin，而不表达HMB45，CD21、CD45、CD20、CD3、CK、CD30、EMA。患者术后恢复良好。

Abstract: Langerhans cell histiocytosis (LCH) is a neoplastic diseases majorly formed by the clonal neoplastic proliferation of Langerhans cells, and single-system which occurs in the lymph nodes is rare. So the clinical misdiagnosis often occurs. In this case, a 45-year-old man was admitted to hospital with a history of fever and a right groin mass for more than one month. Under microscopy, the structure of the lymph node was almost damaged, which was caused by the idiopathic proliferation of Langerhans cell and eosinophils or eosinophilic abscess. The Langerhans cells ranged from medium to relatively large in size and the cytoplasm is rich and eosinophilic. The nuclear was round, oval, bean-like or irregular in shape, showing clear grooves and depressions. Immunohistochemical study showed that the Langerhans cells were strongly positive for S-100 protein, CD1a, CD68, vimentin, and negative for HMB45, CD21, CD45, CD20, CD3, CK, CD30, EMA. The patient was recovered well after surgery.