朗格汉斯组织细胞增生症2例并文献复习

Two cases of langerhans cell histiocytosis

目的:探讨朗格汉斯组织细胞增生症的临床病理特点、鉴别诊断及生物学特征。方法:对2例朗格汉斯组织细胞增生症进行组织学观察和免疫组化染色,并复习相关文献。结果:两例患者分别为成人和儿童,均以头部肿物为首发症状,伴有不同程度的颅骨破坏。手术切除病灶后成人无复发,儿童一个月后颈部又发现肿物并侵犯横纹肌。2例均经病理学诊断为朗格汉斯组织细胞增生症。结论:朗格汉斯组织细胞增生症是一种影响网状内皮系统的疾病,主要以朗格汉斯细胞浸润为主,组织学为良性,但其生物学行为却呈侵袭性、破坏性,可累及骨、肺等多个器官。

Objective：To investigate the clinicopathologic features ,biological characteristics of langerhans cell his- tiocytosis（LCH） and its differentiated diagnosis. Metbods：Histopathologic,immunohistochemieal stainings and ultra- structural observation were analyzad in two cases of LCH. Results：One case was adult and the other was child, both had goiter in their heads and different dgree of osteoclasia. After operation of focus of infection,the adult was health, but the child found a goiter in his transverse part after one month. They were diagnosed as langerhans cell histiocyto- sis. Condusion：Langerhans cell histioeytosis is a disease,which effects endothelial system and depends on langerhans cell,its histology is innocence,but its biological behaviour is infiltrative,devastating,and it can involve bone,lung and so on.