title: Clinical features and treatment of natural killer/T cell lymphoma associated with hemophagocytic syndrome: comparison with other T cell lymphoma associated with hemophagocytic syndrome.
abstract: Natural killer (NK)/T cell lymphoma associated with hemophagocytic syndrome (NK/T-LAHS) is a rare and life-threatening disease. The clinical features and overall survival of NK/T cell and other T cell lymphomas associated with hemophagocytic syndrome remain uncertain. We retrospectively reviewed the clinical records of 15 patients with NK/T-LAHS and 14 patients with other T-LAHS from December 2008 to June 2013. Patients with NK/T cell lymphoma had a higher International Prognostic Index (p = 0.045) and were more likely to be positive for Epstein-Barr virus (p = 0.025) than those with T cell lymphoma. The level of aspartate aminotransferase (AST) was significantly higher than that of alanine aminotransferase (ALT) in the NK/T-LAHS group (p = 0.005), as well as in the T-LAHS group (p = 0.019). The level of direct bilirubin (DBIL) was more likely to be elevated than that of indirect bilirubin (IBIL) in patients with NK/ T-LAHS (p = 0.047), while there was no significant difference in the T-LAHS group (p = 0.124). The median survival time for patients with NK/T and T cell lymphoma was 28 and 33 days, respectively (p = 0.726). However, in the NK/T-LAHS group, the median survival time of patients treated with and without pegaspargase was 116 and 15 days, respectively (p = 0.003). Our results suggest that patients with NK/T-LAHS are at higher risk and suffer a worse prognosis. However, the use of pegaspargase could benefit patients and lead to long-term overall survival.
authors.affiliation: Department of Oncology, the First Affiliated Hospital of Zhengzhou University , Zhengzhou, Henan , China.
authors.forename: Lijuan
authors.initials: L
authors.lastname: Han
authors.forename: Ling
authors.initials: L
authors.lastname: Li
authors.forename: Jingjing
authors.initials: J
authors.lastname: Wu
authors.forename: Xin
authors.initials: X
authors.lastname: Li
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.forename: Xinhua
authors.initials: X
authors.lastname: Wang
authors.forename: Xiaorui
authors.initials: X
authors.lastname: Fu
authors.forename: Wang
authors.initials: W
authors.lastname: Ma
authors.forename: Zhenchang
authors.initials: Z
authors.lastname: Sun
authors.forename: Xudong
authors.initials: X
authors.lastname: Zhang
authors.forename: Yu
authors.initials: Y
authors.lastname: Chang
authors.forename: Shuxia
authors.initials: S
authors.lastname: Guo
authors.forename: Mingzhi
authors.initials: M
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality; D054391:Lymphoma, Extranodal NK-T-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality; D016399:Lymphoma, T-Cell / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: NK/T cell lymphoma; clinical features; hemophagocytic syndrome (HPS); pegaspargase; treatment