Marginal Zone Lymphoma (MZL)

Overview


Plain-Language Overview

Marginal Zone Lymphoma (MZL) is a type of cancer that affects the immune system, specifically a group of white blood cells called B lymphocytes. It usually starts in the marginal zone of lymphoid tissues, which are areas in the body where immune cells gather to fight infections. This cancer can develop in lymph nodes or in other organs like the stomach, lungs, or spleen. People with MZL may experience symptoms such as swollen lymph nodes, fatigue, or unexplained weight loss. The disease affects the body's ability to fight infections properly and can sometimes cause complications if it spreads.

Clinical Definition

Marginal Zone Lymphoma (MZL) is a group of indolent B-cell non-Hodgkin lymphomas arising from the marginal zone of lymphoid tissues. It is characterized by the clonal expansion of mature B cells that typically express CD20 and lack markers of other lymphoma subtypes. MZL is subdivided into three main types: extranodal MZL of mucosa-associated lymphoid tissue (MALT lymphoma), nodal MZL, and splenic MZL. The pathogenesis often involves chronic antigenic stimulation, such as infection with Helicobacter pylori in gastric MALT lymphoma or autoimmune disorders. Clinically, MZL presents with localized or disseminated lymphadenopathy and may cause organ-specific symptoms depending on the site of involvement. It is generally slow-growing but can transform into a more aggressive lymphoma.

Inciting Event

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Clinical Presentation


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Diagnostic Workup


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Pathophysiology


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