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March 25, 2026American Journal of Hematology4 citations

Marginal Zone Lymphoma: 2026 Update on Diagnosis and Management

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WNWan Danial NoorJSJohn F. SeymourCCCalvin Cheah

Key Points

  • To update clinical practices for the diagnosis and management of marginal zone lymphoma (MZL).
  • Reviewed frontline treatment strategies for localized and advanced-stage MZL.
  • Analyzed the role of local therapies and systemic immunotherapy approaches.
  • Considered emerging data on novel therapies including cBTKi and antibody-drug conjugates.
  • Localized MZL favors local treatments like radiotherapy and rituximab monotherapy.
  • Advanced-stage MZL typically requires systemic therapies including immunotherapy and chemotherapy.
  • Emerging therapies such as cBTKi demonstrate potential in relapsed/refractory cases.

Abstract

Frontline treatment of MZL varies between localized disease and advanced-stage diseases. The former favor the use of local therapies such as radiotherapy and anti-microbial in certain subtypes with a selective role of rituximab monotherapy. The latter requires systemic immunotherapy ± chemotherapy with emerging data for cytotoxic-free and covalent Bruton tyrosine kinase inhibitor (cBTKi)-based regimens. cBTKi and immunomodulators are established in relapsed/refractory MZL, while data for antibody-drug conjugates, T-cell engagers, and potentially CAR T-cell therapies are maturing.

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Cite This Study

Noor et al. (2026) studied this question.

synapsesocial.com/papers/69c37be2b34aaaeb1a67eb08https://doi.org/10.1002/ajh.70289
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