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自然杀伤/T细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者预后的多因素分析。

Multivariate analysis of prognosis for patients with natural killer/T cell lymphoma-associated hemophagocytic lymphohistiocytosis.

作者信息

Jin Zhili, Wang Yini, Wang Jingshi, Wu Lin, Pei Ruijun, Lai Wenyuan, Wang Zhao

机构信息

a Department of Hematology , Beijing Friendship Hospital, Capital Medical University , Beijing , People's Republic of China.

出版信息

Hematology. 2018 May;23(4):228-234. doi: 10.1080/10245332.2017.1385191. Epub 2017 Oct 6.

DOI:10.1080/10245332.2017.1385191
PMID:28982299
Abstract

OBJECTIVE

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.

METHODS

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.

RESULTS

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.

CONCLUSION

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.

摘要

目的

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。

方法

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。

结果

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。

结论

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。

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