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An international analysis evaluating frontline bendamustine with rituximab in extranodal marginal zone lymphoma
Journal article   Open access   Peer reviewed

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

Juan Pablo Alderuccio, Luca Arcaini, Marcus P. Watkins, Anne W. Beaven, Geoffrey Shouse, Narendranath Epperla, Michele Spina, Alexandra Stefanovic, Jose Sandoval-Sus, Pallawi Torka, …
Blood advances, Vol.6(7), pp.2035-2044
04/12/2022
DOI: 10.1182/bloodadvances.2021006844
PMCID: PMC9006265
PMID: 35196377
url
https://doi.org/10.1182/bloodadvances.2021006844View
Published (Version of record) Open Access

Abstract

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.
Hematology Life Sciences & Biomedicine Science & Technology

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