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Clinical features and survival of extranodal natural killer/T cell lymphoma with and without hemophagocytic syndrome

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Abstract

Extranodal natural killer (NK)/T cell lymphoma-associated hemophagocytic syndrome (HPS) (NK/T-LAHS) is a heterogeneous and life-threatening disease, which warrants investigation of its risk factors and clinical features. We retrospectively analyzed the clinical records of 202 patients with extranodal NK/T cell lymphoma and compared the characteristics and survival of extranodal NK/T cell lymphoma patients with and without HPS. The cumulative incidence of NK/T-LAHS was 11.4 % (23/202). In a multivariate logistic regression model, younger age (p = 0.012), bone marrow involvement (p = 0.012), and reduced serum albumin (p < 0.001) were independent risk factors for developing HPS in patients with extranodal NK/T cell lymphoma. The survival of extranodal NK/T cell lymphoma patients was aggravated when complicated with HPS, with an overall 2-year survival of 72.1 and 30.4 %, respectively (p < 0.001). Six patients with HPS onset at lymphoma diagnosis tended to have a poor performance status (p = 0.040), while the rate of elevated bilirubin was significantly higher in 17 patients with HPS onset at lymphoma relapse (p = 0.045). After HPS onset, treatment response was poor (response rate, 17.4 %) and survival was dismal with a median of 26 days. Univariate analysis showed that patients with lactate dehydrogenase >1000 U/L (p = 0.048) and disseminated intravascular coagulation (p = 0.004) had shorter survival time. Extranodal NK/T cell lymphoma was frequently complicated with HPS, and survival was discouraging in this circumstance. Intensive chemotherapy regimens including l-asparaginase or pegaspargase and allogeneic stem cell transplantation should be investigated.

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Authors and Affiliations

  1. Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Department of Lymphoma, Peking University Cancer Hospital & Institute, No.52 Fucheng Road, Haidian District, Beijing, 100142, China

    Jing Jia, Yuqin Song, Ningjing Lin, Weiping Liu, Lingyan Ping, Wen Zheng, Xiaopei Wang, Yan Xie, Meifeng Tu, Chen Zhang, Zhitao Ying, Lijuan Deng, Ning Ding & Jun Zhu

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  1. Jing Jia
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  2. Yuqin Song
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Correspondence to Jun Zhu.

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Jia, J., Song, Y., Lin, N. et al. Clinical features and survival of extranodal natural killer/T cell lymphoma with and without hemophagocytic syndrome. Ann Hematol 95, 2023–2031 (2016). https://doi.org/10.1007/s00277-016-2805-9

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  • DOI: https://doi.org/10.1007/s00277-016-2805-9

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