1概述噬血细胞淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)是一组单核-巨噬细胞系统反应性增生而发生过度的炎症反应,造成多组织器官损害的综合征。目前,其病因及发病机制未完全阐明。促炎症反应的细胞因子不断生成,巨噬细胞和细胞毒性T淋巴细胞浸润多组织是其最显著特点[1]。1.1发病率及发病机制HLH可分为家族性HLH 1 Overview Hemophagocytic lymphohistiocytosis (HLH) is a group of mononuclear-macrophage system hyperplasia and hyperinflammatory reaction, resulting in multiple organ damage syndrome. At present, its etiology and pathogenesis are not fully elucidated. The pro-inflammatory cytokines continue to be produced. The most prominent feature of macrophages and cytotoxic T-lymphocytes infiltration is multi-tissue [1]. 1.1 incidence and pathogenesis HLH can be divided into familial HLH