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.

journal_name

Leuk Lymphoma

journal_title

Leukemia & lymphoma

authors

Han L,Li L,Wu J,Li X,Zhang L,Wang X,Fu X,Ma W,Sun Z,Zhang X,Chang Y,Guo S,Zhang M

doi

10.3109/10428194.2013.876629

subject

Has Abstract

pub_date

2014-09-01 00:00:00

pages

2048-55

issue

9

eissn

1042-8194

issn

1029-2403

journal_volume

55

pub_type

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