Session: 625. T Cell, NK Cell, or NK/T Cell Lymphomas: Clinical and Epidemiological: Poster I
Hematology Disease Topics & Pathways:
Research, Clinical Research, Real-world evidence
Methods: Data of 224 patients, including 108 ANKL patients diagnosed between 2000 and 2021 from ANKL22 study (UMIN 000046096) and 116 advanced-stage ENKL patients diagnosed between 2014 and 2021 from NKEA-Next project (UMIN 000046300), were analyzed. Diagnosis was made by considering the distribution of the disease, clinical course and EBV status, based on the World Health Organization classification.
Results: The median age was 49 years in ANKL patients and 60 years in ENKL patients (P < 0.001). Among 116 ENKL patients, 5 were stage III and 111 were stage IV. There was no sex predilection observed in either group. B symptoms were more frequently observed in ANKL patients (86% vs. 53%; P < 0.001), along with poorer performance status (PS) at diagnosis (ECOG PS 2-4: 46% vs. 34%; P = 0.04). In terms of the site of involvement, the common extranodal sites were bone marrow (100%), spleen (84%), liver (70%), and peripheral blood (57%) for ANKL, and nasal cavity (56%), bone/bone marrow (45%), and skin (42%) for ENKL. The frequency of lymph node involvement was similar between both subtypes (40% for ANKL and 36% for ENKL). Pancytopenia, particularly thrombocytopenia, was more prevalent in ANKL patients than in ENKL patients (median platelet count, 4.6×104/μL vs. 19.5×104/μL; P < 0.001). Soluble IL-2 receptor level was significantly higher in ANKL patients than in ENKL patients (median, 8,274 U/dL vs. 1,408U/dL; P < 0.001). Among patients tested for EBV DNA positivity in peripheral blood, approximately 90% were detectable in both groups (89% for ANKL and 92% for ENKL; P = 0.51). Overall survival (OS) was significantly worse for ANKL than advanced-stage ENKL, with a 2-year OS of 18.3% and 37.7%, respectively (P < 0.001). This difference persisted when comparing ANKL and ENKL stage IV (2-year OS: 35.6% for ENKL stage IV; P < 0.001). Moreover, the prognosis for ANKL was significantly worse than that for ENKL with bone/bone marrow involvement (2-year OS: 18.3% vs. 37.9%; P = 0.003). Regarding first-line treatment, SMILE chemotherapy was the most commonly used for both subtypes (42% for ANKL and 55% for ENKL). The 2-year OS of patients treated with SMILE was 22.8% for ANKL and 57.1% for ENKL, respectively (P < 0.001). Fifty-two ANKL patients (48%) underwent hematopoietic stem cell transplantation (HSCT) (allogeneic HSCT 49, autologous HSCT 3), and 45 ENKL patients (39%) underwent HSCT (allogeneic HSCT 26, autologous HSCT 19). The prognosis of ANKL patients who underwent HSCT was significantly worse than that of ENKL patients who underwent HSCT (2-year OS: 36.3% vs. 63.7%; P = 0.008), but significantly better than that of ENKL patients who did not undergo HSCT (2-year OS: 18.0%; P = 0.03). Among patients undergoing allogeneic HSCT, ANKL patients who underwent allogeneic HSCT had worse OS than ENKL patients who underwent allogeneic HSCT (2-year OS: 36.4% vs. 55.2%; P = 0.06).
Conclusion: The present study demonstrated differences in clinical characteristics and prognosis between ANKL and advanced-stage ENKL patients. Despite being younger, ANKL patients have a worse prognosis and require more effective treatments, including allogeneic HSCT. Further studies are warranted to elucidate the differences between these diseases.
Disclosures: Fujimoto: Chugai: Honoraria; Meiji Seika: Honoraria; Nippon Kayaku: Honoraria; Sanofi: Honoraria. Miyazaki: Incyte: Honoraria; Ono Pharmaceuticals: Honoraria; SymBio Pharmaceuticals: Honoraria; Kyowa Kirin: Honoraria, Research Funding; Chugai: Honoraria, Research Funding; Otsuka: Research Funding; Takeda: Research Funding; AstraZeneca: Honoraria; Janssen: Honoraria; Novartis: Honoraria; Genmab: Honoraria; Asahi Kasei: Honoraria; Bristol Myers Squibb: Honoraria; Meiji Seika: Honoraria; Zenyaku Kogyo: Research Funding; Abbvie: Honoraria; Sumitomo Pharma: Research Funding. Maeda: AbbVie: Honoraria; Nippon Shinyaku: Honoraria; Novartis: Honoraria; Ono: Honoraria; Sanofi: Honoraria; Daiichi Sankyo: Honoraria; Takeda: Honoraria; AstraZeneka: Honoraria; Janssen: Honoraria; Chugai: Honoraria; Bristol Myers Squibb: Honoraria. Yakushijin: Asahi Kasei Pharma: Honoraria; AstraZeneca: Honoraria; Novartis: Honoraria; Janssen: Honoraria; Pfizer: Honoraria; Jazz Pharmaceuticals: Honoraria; Otsuka Pharmaceutical: Honoraria; Chugai Pharmaceutical: Research Funding; AbbVie: Honoraria, Research Funding; Nippon Shinyaku: Honoraria. Munakata: CHUGAI PHARMACEUTICAL, Janssen Pharmaceutical, ONO PHARMACEUTICAL, Kyowa Kirin, Genmab, NIPPON SHINYAKU: Research Funding; Mundipharma, Takeda Pharmaceutical, ONO PHARMACEUTICAL, Eisai, CHUGAI PHARMACEUTICAL, Bristol-Myers Squibb, AstraZeneca, NIPPON SHINYAKU, Gilead Sciences, Nippon Kayaku, MSD, Janssen Pharmaceutical, AbbVie GK, Amgen: Honoraria. Fujino: Janssen Pharmaceutical K.K. Chugai Pharmaceutical AbbVie Astellas Pharma Kyowa Kirin NIPPON SHINYAKU: Speakers Bureau. Fukuhara: Eisai: Honoraria; Gilead: Honoraria; Eli Lilly: Honoraria; Novartis: Honoraria; Nippon kayaku: Honoraria; LOXO Oncology: Research Funding; Incyte and Takeda: Research Funding; Janssen: Honoraria; Meiji Seika: Honoraria; Soreisia: Honoraria; Ono: Honoraria; Takeda: Honoraria, Research Funding; Bristol Myers Squibb: Honoraria; Chordia Therapeutics: Research Funding; Chugai Pharma: Honoraria, Research Funding; AbbVie: Honoraria, Research Funding; AstraZeneca: Honoraria; Genmab: Honoraria, Research Funding; Kyowa Kirin: Honoraria, Research Funding. Takizawa: Asahi Kasei: Research Funding; Janssen: Consultancy, Honoraria, Research Funding; AstraZeneca: Consultancy, Honoraria, Research Funding; AbbVie: Consultancy, Honoraria, Research Funding; Novartis: Honoraria, Research Funding; Chugai: Honoraria, Research Funding; Kyowa Kirin: Honoraria, Research Funding; Nippon Kayaku: Honoraria, Research Funding; Mitsubishi Tanabe: Research Funding; Eli Lilly: Research Funding. Maruyama: Ono: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Janssen: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Eisai: Honoraria, Research Funding; Chugai: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Kyowa Kirin: Honoraria, Research Funding; MSD: Honoraria, Research Funding; Zenyaku: Honoraria, Research Funding; Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Symbio: Honoraria, Research Funding; Takeda: Honoraria, Research Funding; AbbVie: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; AstraZeneca: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; BMS: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Genmab: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Honoraria, Research Funding; Otsuka: Research Funding; Taiho: Research Funding; Pfizer: Membership on an entity's Board of Directors or advisory committees, Research Funding; Astellas: Research Funding; Nippon Shinyaku: Honoraria; Mundipharma: Honoraria. Goto: Kyowa Kirin: Research Funding; Bristol-Myers Squibb: Research Funding; Chugai: Honoraria; Sanofi: Research Funding; SymBio: Research Funding. Sakai: Kyowa Kirin: Honoraria, Research Funding; Chugai: Honoraria, Research Funding; Takeda: Honoraria; AstraZeneca: Honoraria; SymBio: Honoraria; Janssen: Honoraria; CSL Behring: Honoraria; Eisai: Honoraria; Nippon Shinyaku: Honoraria; Bristol Meyer Squibb: Honoraria; Mundipharma: Honoraria; Meiji Seika: Honoraria; Towa yakuhin: Honoraria; Nihon Medi-Physics: Honoraria; Sanofi: Honoraria. Asano: Takeda Pharmaceutical Company Limited: Honoraria. Yamaguchi: Genmab: Consultancy, Research Funding; BeiGene: Consultancy; Nihon Servier: Consultancy; AstraZeneca: Research Funding; Chugai: Honoraria, Research Funding; Incyte: Research Funding; AbbVie: Honoraria, Research Funding; Kyowa Kirin: Honoraria; Bristol Myers Squibb: Honoraria; Janssen: Honoraria; Meiji Seika: Honoraria; MSD: Honoraria; Nippon Shinyaku: Honoraria; SymBio: Honoraria; Takeda: Honoraria; Eisai: Honoraria. Suzuki: Chugai, Kyowa-Kirin, Shionogi, Taiho, Eisai, Ohtsuka: Research Funding; Chugai, Kyowa-Kirin, AbbVie, Bristol-Meyers Squibb, Eisai, Ohtsuka, MSD, Janssen, Takeda, Meiji-Seika, Novartis, AstraZeneca: Honoraria.