噬血细胞综合征（ HPS）不是一种独立的疾病，而是一组临床综合征，临床特征是持续性发热、肝脾大、血细胞减少及在骨髓、肝脾和淋巴结的组织细胞噬血现象。 HPS 分为原发性和继发性两种类型。恶性肿瘤特别是恶性淋巴瘤是引起成人继发性 HPS的主要原因。结外 NK／T细胞淋巴瘤相关性HPS（ NK／T－LAHS）临床上少见且致死率高，其临床表现不典型，早期诊断困难，治疗效果差，预后不佳，值得临床医师重视。“,”Hemophagocytic syndrome(HPS) is not an independent disease but a clinical syndrome with a group of clinical features such as persistent fever,hepatosplenomegaly,cytopenia,and hemophagocytosis in bone marrow,liver,spleen,and lymphonode.HPS can be classified into primary and secondary types.Malig-nancies,particularly malignant lymphoma,are the leading causes of HPS in adults.Extranodal NK/T-cell lymphoma-associated HPS ( NK/T-LAHS ) is a rare but life-threatening desease.Due to atypical clinical manifestations,difficulties in early diagnosis, poor efficacy and poor prognosis, special attention to NK/T-LAHS should be paid by the clinicians.