Luis Malpica1, Daniel J Enriquez2, Denisse A Castro3,4, Camila Peña5, Henry Idrobo6, Lorena Fiad7, Maria Prates7, Victoria Otero8, Mirna Biglione9, Milagros Altamirano10, Gustavo Sandival-Ampuero2, Ursula Aviles-Perez11, Kelly Meza12, Laura Aguirre-Martinez13, Nancy Cristaldo8, Juan L Maradei14, Luciana Guanchiale15, Pablo Soto16, Jose L Viñuela17, Maria E Cabrera5, Sally Rose Paredes3,4, Eloisa Riva18, Marcos Di Stefano19, Andrea Noboa20, Juan A Choque21, Myrna Candelaria22, Alana Von Glasenapp23, Fabiola Valvert24, Maria A Torres-Viera25, Jorge J Castillo26, Juan Carlos Ramos27, Luis Villela28, Brady E Beltran3,4. 1. Division of Cancer Medicine, Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX. 2. Departamento de Oncologia Medica, Instituto Nacional de Enfermedades Neoplasicas, Lima, Peru. 3. Departamento de Oncología y Radioterapia, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru. 4. Centro de Investigación de Medicina de Precisión, Universidad de San Martin de Porres, Lima, Peru. 5. Hematology Section, Hospital Del Salvador, Santiago, Chile. 6. Hospital Universitario del Valle, Cali, Colombia. 7. Hematología, Hospital Italiano de La Plata, La Plata, Argentina. 8. Sección Hematología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina. 9. Instituto de Investigaciones Biomédicas en Retrovirus y SIDA (INBIRS) UBA-CONICET, Buenos Aires, Argentina. 10. Hospital Guillermo Almenara, Lima, Peru. 11. Universidad Nacional Federico Villareal, Lima, Peru. 12. Department of Pediatrics, Weill Cornell Medicine, New York, NY. 13. Facultad de Salud, Universidad del Valle, Cali, Colombia. 14. Servicio de Hematologia, Hospital Municipal Emilio Ferreyra, Necochea, Buenos Aires, Argentina. 15. Hospital Privado Universitario de Córdoba, Cordoba, Argentina. 16. Hematology Section, Hospital de Puerto Montt, Puerto Montt, Chile. 17. Hematology Section, Hospital Sótero de Rio, Santiago de Chile, Chile. 18. Cátedra de Hematología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay. 19. Hospital Solca Quito, Hospital de los Valles, Universidad San Francisco de Quito, Quito, Ecuador. 20. Servicio de Hematologia, Instituto Oncológico Nacional Dr. Juan Tanca Marengo, Guayaquil, Ecuador. 21. Hospital de Especialidades Materno Infantil-Caja Nacional de Salud, La Paz, Bolivia. 22. Research Division, Instituto Nacional de Cancerología, Mexico City, Mexico. 23. Department of Hematology, Instituto de Prevision Social, Asuncion, Paraguay. 24. Liga Nacional Contra el Cancer, Instituto de Cancerología-INCAN, Ciudad de Guatemala, Guatemala. 25. Universidad Central de Venezuela, Caracas, Venezuela. 26. Bing Center for Waldenström Macroglobulinemia, Dana Farber Cancer Institute, Boston, MA. 27. Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL. 28. Universidad Del Valle de Mexico, Campus Hermosillo, Hospital Fernando Ocaranza del ISSSTE, Sonora, Mexico.
Abstract
PURPOSE: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease caused by the human T-cell leukemia virus type 1. Real-world data of ATLL in Latin America are lacking. PATIENTS AND METHODS: We analyzed patients with ATLL (acute, lymphomatous, chronic, and smoldering) encountered in 11 Latin American countries between 1995 and 2019. Treatment response was assessed according to the 2009 consensus report. Survival curves were estimated using the Kaplan-Meier method and log-rank test. RESULTS: We identified 253 patients; 226 (lymphomatous: n = 122, acute: n = 73, chronic: n = 26, and smoldering: n = 5) had sufficient data for analysis (median age 57 years). Most patients with ATLL were from Peru (63%), Chile (17%), Argentina (8%), and Colombia (7%). Hypercalcemia was positively associated with acute type (57% v lymphomatous 27%, P = .014). The median survival times (months) were 4.3, 7.9, 21.1, and not reached for acute, lymphomatous, chronic, and smoldering forms, with 4-year survival rates of 8%, 22%, 40%, and 80%, respectively. First-line zidovudine (AZT)-interferon alfa (IFN) resulted in an overall response rate of 63% (complete response [CR] 24%) for acute. First-line chemotherapy yielded an overall response rate of 41% (CR 29%) for lymphomatous. CR rate was 42% for etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone versus 12% for cyclophosphamide, vincristine, doxorubicin, and prednisone-like regimen (P < .001). Progression-free survival at 1 year for acute type patients treated with AZT-IFN was 67%, whereas 2-year progression-free survival in lymphomatous type patients who achieved CR after chemotherapy was 77%. CONCLUSION: This study confirms Latin American ATLL presents at a younger age and has a high incidence of lymphomatous type, low incidence of indolent subtypes, and worse survival rates as compared with Japanese patients. In aggressive ATLL, chemotherapy remains the preferred choice for lymphomatous favoring etoposide-based regimen (etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone), whereas AZT-IFN remains a good first-line option for acute subtype.
PURPOSE:Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease caused by the human T-cell leukemia virus type 1. Real-world data of ATLL in Latin America are lacking. PATIENTS AND METHODS: We analyzed patients with ATLL (acute, lymphomatous, chronic, and smoldering) encountered in 11 Latin American countries between 1995 and 2019. Treatment response was assessed according to the 2009 consensus report. Survival curves were estimated using the Kaplan-Meier method and log-rank test. RESULTS: We identified 253 patients; 226 (lymphomatous: n = 122, acute: n = 73, chronic: n = 26, and smoldering: n = 5) had sufficient data for analysis (median age 57 years). Most patients with ATLL were from Peru (63%), Chile (17%), Argentina (8%), and Colombia (7%). Hypercalcemia was positively associated with acute type (57% v lymphomatous 27%, P = .014). The median survival times (months) were 4.3, 7.9, 21.1, and not reached for acute, lymphomatous, chronic, and smoldering forms, with 4-year survival rates of 8%, 22%, 40%, and 80%, respectively. First-line zidovudine (AZT)-interferon alfa (IFN) resulted in an overall response rate of 63% (complete response [CR] 24%) for acute. First-line chemotherapy yielded an overall response rate of 41% (CR 29%) for lymphomatous. CR rate was 42% for etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone versus 12% for cyclophosphamide, vincristine, doxorubicin, and prednisone-like regimen (P < .001). Progression-free survival at 1 year for acute type patients treated with AZT-IFN was 67%, whereas 2-year progression-free survival in lymphomatous typepatients who achieved CR after chemotherapy was 77%. CONCLUSION: This study confirms Latin American ATLL presents at a younger age and has a high incidence of lymphomatous type, low incidence of indolent subtypes, and worse survival rates as compared with Japanese patients. In aggressive ATLL, chemotherapy remains the preferred choice for lymphomatous favoring etoposide-based regimen (etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone), whereas AZT-IFN remains a good first-line option for acute subtype.