Management of marginal zone lymphomas

Area of Science:

• Hematology
• Oncology

Background:

• Marginal zone lymphomas (MZLs) comprise approximately 7% of B-cell non-Hodgkin lymphomas, with subtypes including extranodal (EMZL), nodal, and splenic (SMZL).
• Diagnostic and staging procedures for MZLs are organ-specific.
• Recent data suggest a reassessed role for positron emission tomography/computed tomography (PET/CT) in routine MZL staging, particularly for localized disease or suspected transformation.

Purpose of the Study:

• To review current diagnostic and therapeutic strategies for Marginal Zone Lymphomas (MZLs).
• To highlight advancements in risk stratification and emerging novel therapies for MZLs.

Main Methods:

• Review of recent literature on Marginal Zone Lymphomas (MZLs).
• Analysis of diagnostic imaging, risk stratification, and treatment outcomes.
• Evaluation of emerging therapeutic agents and their potential impact.

Main Results:

• Risk stratification has improved, linking early progression to worse survival.
• Infections like Helicobacter pylori and Hepatitis C virus are associated with some MZL cases, potentially responding to anti-infective therapy.
• Rituximab-based treatments show favorable results, but novel agents like Bruton's tyrosine kinase (BTK) inhibitors are emerging with promising efficacy in the relapsed setting.

Conclusions:

• The management of MZLs requires tailored diagnostic and staging approaches.
• Infection-related MZLs may respond to targeted anti-infective therapies.
• The therapeutic landscape for MZLs is rapidly evolving with promising novel agents, including BTK inhibitors, phosphatidylinositol 3-kinase (PI3K) inhibitors, CAR T-cells, and bispecific antibodies.