l-asparaginase-based regimens followed by allogeneic hematopoietic stem cell transplantation improve outcomes in aggressive natural killer cell leukemia

• Main Authors: Ki Sun Jung, Su-Hee Cho, Seok Jin Kim, Young Hyeh Ko, Eun-Suk Kang, Won Seog Kim
• Format: Article
• Language: English
• Published: BMC 2016-04-01
• Series: Journal of Hematology & Oncology
• Subjects: ANKL; l-asparaginase; Allogeneic HSCT; Response; Survival
• Online Access: http://link.springer.com/article/10.1186/s13045-016-0271-4
• ISSN: 1756-8722

Abstract Aggressive nature killer cell leukemia (ANKL) is a mature NK-T cell lymphoma with worse prognosis, but optimal treatment is unclear. Therefore, we analyzed the efficacy of l-asparaginase-based regimens for ANKL patients. Twenty-one patients who received dexamethasone, methotrexate, ifosfamide, l-asparaginase, and etoposide (SMILE) or etoposide, ifosfamide, dexamethasone, and l-asparaginase (VIDL) chemotherapy at Samsung Medical Center were selected. The overall response rate for all patients was 33 % (7/21); 38 % (5/13) in SMILE and 40 % (2/5) in VIDL, respectively. The median progression-free survival was 3.9 months (95 % CI 0.0–8.1 months) and median overall survival was 7.0 months (95 % CI 2.3–11.7 months). Treatment response (P = 0.001), hematopoietic stem cell transplantation (HSCT) (P = 0.007) and negative conversion of Epstein-Barr virus (EBV) DNA titer after treatment (P = 0.004) were significantly associated with survival. Thus, l-asparaginase-based regimens followed by allogeneic HSCT seem to improve the outcome for ANKL patients.