结外NK/T细胞淋巴瘤相关嗜血细胞综合征23例回顾性分析

23cases of extranodal NK/T-cell lymphoma associated with reactive haemophagocytic syndrome:A retrospective study

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 1ymphoma associated hemophago-cytic 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〉1000IU／L，较正常参考值升高〉4倍，P=0．018。NK／rr_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,treatment effect and prognosis of extranodal NK/T-cell lymphoma associated hemophagocytic syndrome （NK/T- LAHS）. METHODS： Totally 146 NK/T cell lymphoma patients diagnosed between July 2006 and September 2011 in Kunshan First People＇s Hospital and Affiliated Hospital of Fudan U niversity were included, of which 23 developed NK/T-LAHS. The information of clinical features, laboratory findings, treatments and prognosis of these patients were collected. RESULTS： Of the 23 NK/T- LAHS patients, the median age was 45 years old and 15（65.2%） were male. Nineteen（82.6%） cases originated from nasal cavity,nasal sinuses,and ton sil,while the other 4 （17. 4%/{） cases originated from colon, cervix, or skin （P = 0. 035）. LDH levels were elevated 250 IU/L in all 23 cases at the time of diagnosis, and 10（43. 5%） cases showed elevated LDH levels of more than 1 000 IU/L at the onset of HPS,which predicted a poor prognosis（P=0. 018）. NK/T-LAHS was characterized by fever, pancytopenia, liver disfunction, and hypertriglyceridemia, but only 9 cases （39.1%） were found hemophagocytosis in bone marrow. The median overall survival was 8.5 months,and the median LAHS survival time was 14 days. Chemotherapy and highdose glucocorticoids were not so effect to NK/T-LAHS that almost 100 % cases died soon. CONCLUSIONS： The clinical charac ters of NK/T-LAHS are complex,so the rate of its definite diagnosis at the early stage is low. Some cases even died in a short time after the diagnosis. Most of the patients died of multiorgan failure, infection and bleeding. The original lesion and LDH level may be prognostic factors. There is no effect treatment for NK/T-LAHS and it remains a challenge.