title: Aggressive natural killer cell leukemia: therapeutic potential of L-asparaginase and allogeneic hematopoietic stem cell transplantation.
abstract: We conducted a retrospective Japan-Korea multicenter study to better elucidate the clinicopathologic features and therapeutic modalities for aggressive natural killer cell leukemia (ANKL). A total of 34 patients were analyzed. The median age of the patients was 40 years. Among the patients in the study, four had a history of Epstein-Barr virus-related disorders. Three types of ANKL cells were categorized according to their morphological features. Leukemic cells were below 20% in both peripheral blood and bone marrow of 11 patients. The clinical characteristics and prognoses of these 11 patients did not differ significantly from those of the others. As an initial therapy, l-asparaginase chemotherapy resulted in a better response. A total of six patients received allogeneic hematopoietic stem cell transplantation (HSCT) and two received autologous HSCT, with all in non-complete remission (CR). After HSCT, four with allogeneic and one with autologous HSCT reached CR. Median survival of all patients was 51 days. Median survival for the patients with and without HSCT were 266 and 36 days, respectively. A total of two patients with allogeneic HSCT were alive and in CR. All patients without HSCT died of ANKL. The use of L-asparaginase was indicated as a factor for longer survival (HR 0.33, 95% confidence interval; 0.13-0.83, P = 0.02). Early diagnosis of ANKL, l-asparaginase-based chemotherapy and allogeneic HSCT might lead to improved patient outcomes.
authors.affiliation: Department of Hematology, Shinshu University School of Medicine, Matsumoto, Japan. fumishi@shinshu-u.ac.jp
authors.forename: Fumihiro
authors.initials: F
authors.lastname: Ishida
authors.forename: Young Hyeh
authors.initials: YH
authors.lastname: Ko
authors.forename: Won Seog
authors.initials: WS
authors.lastname: Kim
authors.forename: Junji
authors.initials: J
authors.lastname: Suzumiya
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Isobe
authors.forename: Kazuo
authors.initials: K
authors.lastname: Oshimi
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
authors.forename: Ritsuro
authors.initials: R
authors.lastname: Suzuki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015703:Antigens, CD; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D054066:Leukemia, Large Granular Lymphocytic / Q000188:drug therapy* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D015703:Antigens, CD; D000970:Antineoplastic Agents; D001215:Asparaginase