An international analysis evaluating frontline bendamustine with rituximab in extranodal marginal zone lymphoma

• Published in: Blood advances Vol. 6; no. 7; pp. 2035 - 2044
• Main Authors: Alderuccio, Juan Pablo, Arcaini, Luca, Watkins, Marcus P., Beaven, Anne W., Shouse, Geoffrey, Epperla, Narendranath, Spina, Michele, Stefanovic, Alexandra, Sandoval-Sus, Jose, Torka, Pallawi, Alpert, Ash B., Olszewski, Adam J., Kim, Seo-Hyun, Hess, Brian, Gaballa, Sameh, Ayyappan, Sabarish, Castillo, Jorge J., Argnani, Lisa, Voorhees, Timothy J., Saba, Raya, Chowdhury, Sayan Mullick, Vargas, Fernando, Reis, Isildinha M., Kwon, Deukwoo, Alexander, Jonathan S., Zhao, Wei, Edwards, Dali, Martin, Peter, Cencini, Emanuele, Kamdar, Manali, Link, Brian K., Logothetis, Constantine N., Herrera, Alex F., Friedberg, Jonathan W., Kahl, Brad S., Luminari, Stefano, Zinzani, Pier Luigi, Lossos, Izidore S.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 12.04.2022; American Society of Hematology
• Subjects: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols - adverse effects; Bendamustine Hydrochloride - adverse effects; Clinical Trials and Observations; Herpes Zoster; Humans; Lymphoma, B-Cell, Marginal Zone - pathology; Middle Aged; Rituximab - adverse effects; Young Adult
• ISSN: 2473-9529; 2473-9537; 2473-9537
• DOI: 10.1182/bloodadvances.2021006844

Extranodal marginal zone lymphoma (EMZL) is a heterogeneous non-Hodgkin lymphoma. No consensus exists regarding the standard-of-care in patients with advanced-stage disease. Current recommendations are largely adapted from follicular lymphoma, for which bendamustine with rituximab (BR) is an established approach. We analyzed the safety and efficacy of frontline BR in EMZL using a large international consortium. We included 237 patients with a median age of 63 years (range, 21-85). Most patients presented with Eastern Cooperative Oncology Group (ECOG) performance status 0 to 1 (n = 228; 96.2%), stage III/IV (n = 179; 75.5%), and intermediate (49.8%) or high (33.3%) Mucosa Associated Lymphoid Tissue International Prognosis Index (MALT-IPI). Patients received a median of 6 (range, 1-8) cycles of BR, and 20.3% (n = 48) received rituximab maintenance. Thirteen percent experienced infectious complications during BR therapy; herpes zoster (4%) was the most common. Overall response rate was 93.2% with 81% complete responses. Estimated 5-year progression-free survival (PFS) and overall survival (OS) were 80.5% (95% CI, 73.1% to 86%) and 89.6% (95% CI, 83.1% to 93.6%), respectively. MALT-IPI failed to predict outcomes. In the multivariable model, the presence of B symptoms was associated with shorter PFS. Rituximab maintenance was associated with longer PFS (hazard ratio = 0.16; 95% CI, 0.04-0.71; P = .016) but did not impact OS. BR is a highly effective upfront regimen in EMZL, providing durable remissions and overcoming known adverse prognosis factors. This regimen is associated with occurrence of herpes zoster; thus, prophylactic treatment may be considered. •Bendamustine with rituximab is a highly effective frontline therapy in extranodal marginal zone lymphoma across all extranodal sites.•Bendamustine with rituximab overcomes known poor prognosis features in extranodal marginal zone lymphoma. [Display omitted]