Advances in the Pathogenesis, Diagnosis, Treatment, and Prognosis of Marginal Zone Lymphoma.

in Current treatment options in oncology by Qingyang Zhang, Wenzhe Yan, Heng Li, Hongling Peng

TLDR

  • The study provides a framework for managing marginal zone lymphoma (MZL), an indolent B-cell non-Hodgkin lymphoma, with a personalized and adaptive approach.
  • Treatment decisions should consider subtype, disease burden, symptoms, and actionable genetic or environmental factors.
  • The study highlights the importance of molecular testing at diagnosis and ongoing advancements in understanding the tumor microenvironment for improved outcomes.

Abstract

The management of marginal zone lymphoma (MZL), an indolent B-cell non-Hodgkin lymphoma, requires a personalized and adaptive approach due to its clinical and prognostic heterogeneity. We believe treatment should emphasize a balanced strategy considering the subtype, disease burden, symptoms, and actionable genetic or environmental factors, such as infections or autoimmune diseases. For asymptomatic patients with low tumor burden or disseminated disease, a watch-and-wait approach remains appropriate, given MZL's indolent nature and the risks of overtreatment. Conversely, for symptomatic or high-burden cases, early intervention with chemoimmunotherapy is recommended for effective disease control. Surgery remains essential for both diagnosis and the treatment of localized disease. Incorporating molecular profiling and prognostic models, such as MZL-IPI and POD24, is crucial for decision-making and risk stratification. Testing for infectious agents like Helicobacter pylori or Hepatitis C virus should be standard practice, as eradication therapy offers a targeted, less toxic, and effective option in select patients. With ongoing advancements in understanding dysregulated signaling pathways and the tumor microenvironment, we anticipate novel targeted therapies and combination regimens will further improve outcomes. We advocate for molecular testing at diagnosis to identify actionable biomarkers, particularly for patients with refractory or relapsed disease. Finally, MZL management requires vigilant follow-up with adjustments based on evolving disease features. Treatment decisions should integrate patient preferences, clinical context, and the latest evidence to maximize survival while preserving quality of life.

Overview

  • The study focuses on the management of marginal zone lymphoma (MZL), an indolent B-cell non-Hodgkin lymphoma, with an emphasis on a personalized and adaptive approach.
  • The study highlights the importance of considering subtype, disease burden, symptoms, and actionable genetic or environmental factors in treatment decision-making.
  • The primary objective is to provide a balanced strategy for managing MZL, taking into account the heterogeneity of the disease and the need for tailoring treatment to individual patients.

Comparative Analysis & Findings

  • The study suggests that asymptomatic patients with low tumor burden or disseminated disease may benefit from a watch-and-wait approach, given the indolent nature of MZL and the risks of overtreatment.
  • For symptomatic or high-burden cases, early intervention with chemoimmunotherapy is recommended for effective disease control.
  • Surgery remains essential for diagnosis and treatment of localized disease, and molecular profiling and prognostic models, such as MZL-IPI and POD24, are crucial for decision-making and risk stratification.

Implications and Future Directions

  • The study emphasizes the importance of incorporating molecular testing at diagnosis to identify actionable biomarkers, particularly for patients with refractory or relapsed disease.
  • Ongoing advancements in understanding dysregulated signaling pathways and the tumor microenvironment are expected to lead to novel targeted therapies and combination regimens for improved outcomes.
  • The study advocates for vigilant follow-up with adjustments based on evolving disease features and treatment decision-making that integrates patient preferences, clinical context, and the latest evidence to maximize survival while preserving quality of life.
Read Full Article