Hemophagocytic lymphohistiocytosis ( HLH) is one of the common childhood immune diseases. Infection, including bacteria, Epstein-Barr (EB) virus, protozoon, fungi, chlamydia etc, is the common cause of HLH. HLH is characterized by hemophagocytosis and a reactive process resulting from prolonged and excessive activaition of antigen presenting cells (macrophages, histiocytes) and CD8T cells. According to the 2009 HLH plan, etiological control is necessary for HLH treatment. It is confirmed that the application of VP-16 within the first four weeks can improve HLH prognosis. During induction phase of chemotherapy, the appropriate short-term application of cyclosporine A is beneficial to the recovery of granulocytopenia. The poor prognosis factors include high serum ferritin level, lactate dehydrogenase level, IL-2R level and IFN-7 level, EB infection, young age or age > 30 may increase the mortality of HLH.%噬血细胞淋巴组织增生症(HLH)是儿童期常见的免疫性疾病之一.细菌、EB病毒、原虫、真菌、衣原体等感染是HLH的常见病因.目前认为感染相关HLH的发病机制是由于NK细胞和细胞毒T细胞(CTL)介导的穿孔素依赖的细胞毒作用缺陷所导致的多系统炎症反应,感染激活巨噬细胞、树突状细胞、NK细胞和CTL,导致细胞因子风暴形成.不同的感染类型可针对其病因控制.研究证实4周内积极应用依托泊苷在内的化疗方案能够改善预后,化疗诱导阶段适当短期应用环孢素A有利于粒细胞减少症恢复.预后不良的因素包括高血清铁蛋白和乳酸脱氢酶、IL-2R和IFN-γ升高,EB病毒感染引起的HLH、年龄过小或年龄＞ 30岁可能增加病死率.
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