Jin Zhili, Wang Yini, Wang Jingshi, Wu Lin, Pei Ruijun, Lai Wenyuan, Wang Zhao
a Department of Hematology , Beijing Friendship Hospital, Capital Medical University , Beijing , People's Republic of China.
Hematology. 2018 May;23(4):228-234. doi: 10.1080/10245332.2017.1385191. Epub 2017 Oct 6.
A major cause of hemophagocytic lymphohistiocytosis (HLH) is malignant neoplasms of the blood system, among which NK/T cell lymphoma is one of the most common risk factors. Patients with NK/T cell lymphoma hemophagocytic lymphohistiocytosis (NK/T-LAHS) have a worse prognosis and higher mortality. We aimed to explore the factors that affect the prognosis of NK/T-LAHS.
Clinical data of 42 patients with NK/T-LAHS diagnosed by Beijing Friendship Hospital from June 2008 to June 2016 were analyzed retrospectively. The survival time was counted until 1 August 2016.
For the 42 NK/T-LAHS patients, 1-month survival rate was 48.9%, 2-month survival rate was 36.7%, 3-month survival rate was 28.8%, 6-month survival rate was 23.0%, and 12-month survival rate was 15.4%. NK/T-LAHS patients who underwent allogeneic hematopoietic stem cell transplantation (Allo-HSCT) (p = 0.000), exhibited peripheral blood Epstein-Barr virus (EBV)-positivity (p = 0.004), and achieved overall response (OR) remission after initial induction therapy (p = 0.007) had statistical significance.
NK/T-LAHS is a disease of poor prognosis and high mortality. NK/T-LAHS patients who achieved OR remission after the initial induction therapy had a better prognosis than non-remission patients and Allo-HSCT was an effective way to prolong the survival of NK/T-LAHS patients. However, EBV positivity in peripheral blood was a poor prognostic factor in NK/T-LAHS patients.
噬血细胞性淋巴组织细胞增生症(HLH)的一个主要病因是血液系统恶性肿瘤,其中NK/T细胞淋巴瘤是最常见的危险因素之一。NK/T细胞淋巴瘤噬血细胞性淋巴组织细胞增生症(NK/T-LAHS)患者预后较差,死亡率较高。我们旨在探讨影响NK/T-LAHS预后的因素。
回顾性分析2008年6月至2016年6月在北京友谊医院确诊的42例NK/T-LAHS患者的临床资料。计算生存时间至2016年8月1日。
42例NK/T-LAHS患者中,1个月生存率为48.9%,2个月生存率为36.7%,3个月生存率为28.8%,6个月生存率为23.0%,12个月生存率为15.4%。接受异基因造血干细胞移植(Allo-HSCT)的NK/T-LAHS患者(p = 0.000)、外周血爱泼斯坦-巴尔病毒(EBV)呈阳性的患者(p = 0.004)以及初始诱导治疗后达到完全缓解(OR)的患者(p = 0.007)具有统计学意义。
NK/T-LAHS是一种预后差、死亡率高的疾病。初始诱导治疗后达到OR缓解的NK/T-LAHS患者比未缓解患者预后更好,Allo-HSCT是延长NK/T-LAHS患者生存期的有效方法。然而,外周血EBV阳性是NK/T-LAHS患者预后不良的因素。