郎格罕细胞组织细胞增生症(Langerhans Cell Histiocytosis,LCH)是郎格罕细胞异常增生引起的一组疾病。本病多发生于儿童时期,以婴幼儿时期多见。据报道,英国和爱尔兰每年发病率为4.2/1 000 000。男性发病多于女性,我国男女发病比为(1.4～3.4)∶1。本病病因及发病机制尚不完全清楚。由于临床表现多样,误诊率高。LCH常致多系统受累,预后不佳。现就本病发 Langerhans cell histiocytosis (Langerhans Cell Histiocytosis, LCH) is a group of diseases caused by abnormal proliferation of Langerhans cells. The disease occurred in childhood, more common in infants and young children. It is reported that the annual incidence in the UK and Ireland is 4.2 / 1 000 000. The incidence of men more than women, men and women in our country than (1.4 ~ 3.4): 1. Etiology and pathogenesis of this disease is not yet fully understood. Due to the diversity of clinical manifestations, the misdiagnosis rate is high. LCH often caused by multiple system involvement, poor prognosis. Now on the disease