TY - JOUR
T1 - Treatment and Survival Outcomes of Waldenstrom Macroglobulinemia in Latin American Patients
T2 - A Multinational Retrospective Cohort Study
AU - Riva, Eloísa
AU - Duarte, Patricio José
AU - Valcárcel, Bryan
AU - Remaggi, Guillermina
AU - Murrieta, Ivan
AU - Corzo, Ariel
AU - Del Carpio, Daniel
AU - Peña, Camila
AU - Vásquez, Jule
AU - Bove, Virginia
AU - Teixeira, Larissa
AU - Fleury-Perini, Guilherme
AU - Yantorno, Sebastian
AU - Samánez, César
AU - Lopresti, Sergio
AU - Altamirano, Milagros
AU - Villela, Luis
AU - Ruiz-Arguelles, Guillermo J.
AU - Ruiz-Delgado, Guillermo J.
AU - Montaño, Efreen
AU - Verri, Verónica
AU - Zamora Pérez, Elia
AU - Pérez Jacobo, Fernando
AU - Idrobo, Henry
AU - Martínez-Cordero, Humberto
AU - Beltran, Brady E.
AU - Ramírez, Jhoanna
AU - Castillo, Jorge J.
AU - Malpica Castillo, Luis E.
PY - 2022/8/1
Y1 - 2022/8/1
N2 - 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 MYD88L265P, 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.
AB - 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 MYD88L265P, 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.
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U2 - 10.1200/GO.21.00380
DO - 10.1200/GO.21.00380
M3 - Article
C2 - 35939775
AN - SCOPUS:85135549102
SN - 2378-9506
VL - 8
SP - e2100380
JO - JCO Global Oncology
JF - JCO Global Oncology
ER -