Treatment and Survival Outcomes of Waldenstrom Macroglobulinemia in Latin American Patients: A Multinational Retrospective Cohort Study.

Autor:	Riva E; Hematology Department, Hospital Británico & Hospital de Clinicas, Montevideo, Uruguay., Duarte PJ; Hospital Universitario CEMIC, Buenos Aires, Argentina., Valcárcel B; Department of Epidemiology, Milken Institute School of Public Health, The George Washington University, Washington, DC., Remaggi G; Fundaleu, Buenos Aires, Argentina., Murrieta I; Clínica Ruiz, Puebla, Mexico., Corzo A; Hospital de Clínicas de Buenos Aires, Buenos Aires, Argentina., Del Carpio D; Hospital Edgardo Rebagliati, Lima, Peru., Peña C; Hospital del Salvador, Santiago de Chile, Chile., Vásquez J; Instituto Nacional de Enfermedades Neoplásicas, Lima, Peru., Bove V; Hospital Central de las Fuerzas Armadas, Montevideo, Uruguay., Teixeira L; Hospital Israelita Albert Einstein, San Pablo, Brazil., Fleury-Perini G; Hospital Israelita Albert Einstein, San Pablo, Brazil., Yantorno S; Hospital Italiano de La Plata, La Plata, Argentina., Samánez C; Clinica Oncosalud-AUNA, Lima, Peru., Lopresti S; Hospital Nacional Alejandro Posadas, Buenos Aires, Argentina., Altamirano M; Hospital Guillermo Almenara, Lima, Peru., Villela L; Centro Médico Dr Ignacio Chavez, Hospital Fernando Ocaranza, Universidad del Valle, Mexico City, Mexico., Ruiz-Arguelles GJ; Clínica Ruiz, Puebla, Mexico., Ruiz-Delgado GJ; Clínica Ruiz, Puebla, Mexico., Montaño E; Hospital General de Mexico, Mexico City, Mexico., Verri V; Instituto de Investigaciones Médicas Alfredo Lanari, Buenos Aires, Argentina., Zamora Pérez E; Hospital Regional PEMEX de Ciudad Madero, Ciudad Madero, Mexico., Pérez Jacobo F; Hospital Central Norte PEMEX, Mexico City, Mexico., Idrobo H; Centro Médico Julián Coronel, Cali, Colombia., Martínez-Cordero H; Instituto Nacional de Cancerología, Bogotá, Colombia., Beltran BE; Hospital Nacional Edgardo Rebagliati, Lima, Peru., Ramírez J; Hospital Teodoro Maldonado, Guayaquil, Ecuador., Castillo JJ; Bing Center for Waldenström Macroglobulinemia, Dana Farber Cancer Institute, Boston, MA., Malpica Castillo LE; Division of Cancer Medicine, Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX.
Jazyk:	angličtina
Zdroj:	JCO global oncology [JCO Glob Oncol] 2022 Aug; Vol. 8, pp. e2100380.
DOI:	10.1200/GO.21.00380
Abstrakt:	Purpose: Waldenstrom Macroglobulinemia (WM) is a rare lymphoma with distinct clinical features, and data from Latin American patients are lacking. Therefore, we aim to investigate the clinical, therapy, and outcome patterns of WM in Latin America. Methods: We retrospectively analyzed patients with WM diagnosed between 1991 and 2019 from 24 centers in seven Latin American countries. The study outcomes were overall survival (OS) and progression-free survival (PFS). Results: We identified 159 cases (median age 67 years, male 62%). Most patients (95%) were symptomatic at diagnosis. The International Prognostic Scoring System for WM (IPSSWM) at diagnosis was available in 141 (89%) patients (high-risk 40%, intermediate-risk 37%, and low-risk 23%). Twenty-seven (17%) patients were tested for MYD88 L265P , with 89% (n = 24 of 27) carrying the mutation. First-line and second-line therapies were administered to 142 (89%) and 53 (33%) patients, respectively. Chemoimmunotherapy was the most commonly used first-line (66%) and second-line (45%) approach; only 18 (11%) patients received ibrutinib. With a median follow-up of 69 months, the 5-year OS rate was 81%. In treated patients, the 5-year OS and PFS rates were 78% and 59%, respectively. High-risk IPSSWM at treatment initiation was an independent risk factor for OS (adjusted hazard ratio: 4.73, 95% CI, 1.67 to 13.41, P = .003) and PFS (adjusted hazard ratio: 2.43, 95% CI, 1.31 to 4.50, P = .005). Conclusion: In Latin America, the management of WM is heterogeneous, with limited access to molecular testing and novel agents. However, outcomes were similar to those reported internationally. We validated the IPSSWM score as a prognostic factor for OS and PFS. There is an unmet need to improve access to recommended diagnostic approaches and therapies in Latin America.
Databáze:	MEDLINE
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