目的:探讨结外NK/T细胞淋巴瘤相关嗜血细胞综合征(natural killer/T-cell lymphoma associated hemophagocytic syndrome,NK/T-LAHS)的临床特征、治疗效果和预后因素。方法:收集江苏大学附属昆山医院和复旦大学附属肿瘤医院2006-07-01-2011-09-30收治的NK/T细胞淋巴瘤患者146例,其中发生NK/T-LAHS者23例,回顾性分析其临床特征、实验室检查结果、治疗过程及预后情况。结果:23例NK/T-LAHS患者中位年龄45岁(21~79岁),男15例(65.2%)。19例(82.6%)原发部位为鼻及上呼吸道区域,另4例(17.4%)原发于结肠、宫颈和皮肤,P=0.035。所有患者治疗前血清LDH水平均>250IU/L,高于正常,10例(43.5%)在继发HPS后LDH水平>1 000IU/L,较正常参考值升高>4倍,P=0.018。NK/T-LAHS临床表现多样,主要为发热、外周血细胞减少、肝功能异常和高甘油三酯血症,而骨髓中见到嗜血现象的仅有9例(39.1%)。中位生存期为8.5个月,中位NK/T-LAHS生存时间仅为14d。化疗和大剂量激素冲击效果不佳,死亡率达到100%。结论:NK/T-LAHS临床表现复杂,临床诊断较困难;病情发展快,有些患者在确诊后短时间内死亡,死亡原因多为多脏器衰竭、出血或感染。原发部位及血清LDH水平变化可能是NK/T-LAHS预后不良的主要因素。目前尚缺乏有效治疗手段。 Objective: To investigate the clinical features, therapeutic effects and prognostic factors of extranodal NK / T-cell lymphoma associated hemophagocytic syndrome (NK / T-LAHS). Methods: 146 patients with NK / T cell lymphoma were enrolled from Kunshan Hospital Affiliated to Jiangsu University and Affiliated Tumor Hospital of Fudan University 2006-07-01-2011-09-30 Twenty-three patients with NK / T-LAHS were retrospectively reviewed Analyze its clinical characteristics, laboratory test results, treatment process and prognosis. Results: The median age of 23 patients with NK / T-LAHS was 45 years (21-79 years), 15 were male (65.2%). Nineteen patients (82.6%) had primary nasal and upper respiratory tract and the other four (17.4%) developed primary colon, cervix and skin, P = 0.035. All patients had serum LDH> 250IU / L before treatment, higher than normal, and LDH level> 1 000IU / L after secondary HPS in 10 patients (43.5%), which was 4 times higher than the normal reference value (P = 0.018). NK / T-LAHS has various clinical manifestations, mainly fever, peripheral blood cells, abnormal liver function and hypertriglyceridemia, while only 9 cases (39.1%) have seen the bloodthirsty phenomenon in bone marrow. The median survival was 8.5 months, median NK / T-LAHS survival time was only 14d. Chemotherapy and high-dose hormone impact is not good, the mortality rate reached 100%. Conclusions: The clinical manifestations of NK / T-LAHS are complex and clinical diagnosis is more difficult. The disease develops rapidly. Some patients die shortly after diagnosis. The causes of death are mostly multiple organ failure, bleeding or infection. The change of primary site and serum LDH level may be the main factor of poor prognosis of NK / T-LAHS. Currently there is a lack of effective treatment.