Extranodal NK NK /T‐cell lymphoma, nasal type: Clinical features, outcome, and prognostic factors in 101 cases

摘要

Abstract Objectives We aimed to define the clinical features, outcome, and prognostic factors for extranodal NK /T‐cell lymphoma ( ENKTL ) patients in Taiwan. Methods We retrospectively reviewed 101 ENKTL patients diagnosed between February 1998 and October 2015. Results The median age of 101 patients was 52?years old (range 22‐85); 76.2% of patients were Ann Arbor stage I/ II disease. The 5‐year progression‐free survival ( PFS ) and overall survival ( OS ) were 49.9% and 54.8%, respectively. Patients with log[ EBV ‐ DNA ]?≥?3.8 and bone marrow hemophagocytosis at diagnosis had inferior PFS and OS . Most stage I/ II patients received combined chemoradiotherapy with anthracycline‐containing regimen, with overall response rate of 96.7%, complete response rate 86.9%, 5‐year PFS 65%, and OS 72%. The relapse rate was 29.3% with a short median disease‐free survival of 6.2?months. In advanced stage patients, overall response rate was only 13.6%, with median PFS 2.3?months, and OS 4.8?months. Age?≥?60 ( HR 3.773, 95% CI 1.733‐8.215, P? =?0.001) and stage III / IV ( HR 7.785, 95% CI 2.312‐26.213, P? =?0.001) were unfavorable prognostic factors for PFS and OS by multivariate analyses. Conclusions Age?≥?60 and stage III / IV are independent poor prognostic factors for PFS and OS . Early‐stage ENKTL patients had good response to combined chemoradiotherapy with anthracycline‐containing regimen but with a high relapse rate and short disease‐free survival. Anthracycline‐containing regimen in advanced stage had poor response and dismal outcome.