Prognostic factors for mature natural killer (NK) cell neoplasms: aggressive NK cell leukemia and extranodal NK cell lymphoma,nasal type |
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| Institution: | 1. Department of HSCT Data Management, Nagoya University School of Medicine, Nagoya;2. Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka;3. Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu;4. Department of Pathology, Nagoya University Graduate School of Medicine, Nagoya;5. Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai;6. Division of Hematology, Institute of Clinical Medicine, University of Tsukuba, Tsukuba;7. First Department of Pathology, Fukushima Medical University, Fukushima;8. Department of Hematology and Oncology, Nagoya University Graduate School of Medicine, Nagoya;9. Department of Pathology;10. Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama;11. Department of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya;12. Department of Pathology, Nagoya Daini Red Cross Hospital, Nagoya;13. Department of Dermatology, Miyazaki University School of Medicine, Miyazaki;14. Department of Dermatology, Niigata University Graduate School of Medicine, Niigata;15. Department of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka;16. Department of Hematology, Juntendo University, Tokyo, Japan |
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| Abstract: | Background: Patients with natural killer (NK) cell neoplasms, aggressive NK cell leukemia (ANKL) and extranodal NK cell lymphoma, nasal type (ENKL), have poor outcome. Both diseases show a spectrum and the boundary of them remains unclear. The purpose of this study is to draw a prognostic model of total NK cell neoplasms.Patients and methods: We retrospectively analyzed 172 patients (22 with ANKL and 150 with ENKL). The ENKLs consisted of 123 nasal and 27 extranasal (16 cutaneous, 9 hepatosplenic, 1 intestinal and 1 nodal) lymphomas.Results: Complete remission rate for ENKL was 73% in stage I, but 15% in stage IV, which was consistent with that for ANKL (18%). The prognosis of ENKL was better than that of ANKL (median survival 10 versus 1.9 months, P < 0.0001) but was comparable when restricted to stage IV cases (4.0 months, P = 0.16). Multivariate analysis showed that four factors (non-nasal type, stage, performance status and numbers of extranodal involvement) were significant prognostic factors. Using these four variables, an NK prognostic index was successfully constructed. Four-year overall survival of patients with zero, one, two and three or four adverse factors were 55%, 33%, 15% and 6%, respectively.Conclusion: The current prognostic model successfully stratified patients with NK cell neoplasms with different outcomes. |
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