中文摘要：

噬血细胞综合征（hemophagoeytie lymphohistiocytosis, HLH ）是由感染、风湿免疫和恶性肿瘤等多种致病因素引发的高炎症反应综合征。其中恶性肿瘤相关的噬血细胞综合征（malignancy-associated hemophagocytic lymphohistiocytosis, MAHS）起病凶险、误诊率和死亡率均较高，之前对其诊断和治疗尚未达成共识。为了更好地指导临床，2015年国际组织细胞协会（HLH）对MAHS的认识达成专家共识。本文将结合近些年MAHS的研究进展和2015年MAHS专家共识做一综述。

英文摘要：

Hemophagocytic lymphohistiocytosis （HLH） is a hyperinflammatory response syndrome induced by various pathogenic fac- tors, such as infection, rheumatic disease, and malignancy. Malignancy-associated hemophagocytic lymphohistiocytosis （MAHS） is a life-threatening disease with high rates of misdiagnosis and mortality. The diagnosis and treatment of MAHS have not been standard- ized until consensus recommendations were developed by the Study Group on Hemophagocytic Lymphohistiocytosis subtypes of the Histiocyte Society in 2015. This article reviews the consensus recommendations and recent advances in MAHS.