Marginal Zone Lymphoma

Overview

Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Lymphoma occurs when lymphocytes, a type of white blood cell, grow abnormally. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood or other organs, and can accumulate to form tumors.

Marginal zone lymphoma is a group of indolent (slow-growing) B-cell lymphomas, which account for approximately 12 percent of all B-cell lymphomas. The median age for diagnosis is 65.

Subtypes

There are three types of marginal zone lymphoma:

Extranodal marginal zone lymphoma of mucosa-associated lymphatic tissue (MALT) is the most common form of marginal zone lymphoma. It occurs outside the lymph nodes, such as the stomach, small intestine, salivary gland, thyroid, eyes and lungs. MALT is divided into two categories: gastric MALT, which develops in the stomach, and non-gastric MALT, which develops outside of the stomach. This form of lymphoma makes up approximately 9 percent of all B-cell lymphomas.

In many cases of MALT lymphoma, there is a previous medical history of inflammation or autoimmune disorders. For example, Helicobacter pylori (H. pylori), a microbial pathogen linked to chronic gastritis, has been associated with a significant portion of gastric MALT patients.

Nodal marginal zone lymphoma (sometimes called monocytoid B-cell lymphoma) occurs within the lymph nodes and makes up approximately 2 percent of all B-cell lymphomas.

Splenic marginal zone lymphoma occurs mostly in the spleen and blood. It has been associated with Hepatitis C. This form of lymphoma makes up approximately 1 percent of all B-cell lymphomas. 

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