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A strange journey of external marginal lymphomas: Why do they appear in other organs?
External marginal zone lymphoma (MZL) is a heterogeneous lymphoma population that mainly originates from the malignant transformation of B cells in marginal zones.These B cells are natural lymphocytes that are often able to rapidly develop IgM antibody immune responses to infected pathogens and damaged tissues.These cells are produced and mature in secondary lymphatic follicles and then enter the marginal area of mucosal-associated lymphoid tissue (MALT), spleen, or lymph nodes.With the World Health Organization's classification in 2016, MZL is classified into three types, including many lymphomas that develop in MALT tissues, called peripheral MALT lymphoma or simply MALT lymphoma.
MZL accounts for about 5-17% of all non-Hodgkin lymphomas, while peripheral, spleen and lymph node forms account for about 50-70%, 20% and 10%.
Depending on the location of the tumor, peripheral MZL usually occurs in external organs such as the stomach, small intestine, and skin. These lymphomas are therefore closely related to infection or autoimmune diseases.Here, the pathophysiological and histological characteristics of peripheral MZL are shown to provide clues for the development of lymphoma.For example, nodules or diffuse cell distributions that are visible to the naked eye, and the histological characteristics of tumor cells show the malignant evolution of B cells.
Pathophysiology
The evolution of lymphomas in outer marginal zones usually involves a variety of factors, among which chronic inflammation is triggered by infection or autoimmune response, further promoting abnormal proliferation of B cells.During this process, not only chromosomal abnormalities and gene mutations occur, but also lead to abnormal gene expression.Abnormal development of these biochemical pathways directly promotes the malignancy of B cells and leads to the formation of MZL.
Histological characteristics
In histological examination, the lesions of lymphoma in the outer marginal zone were shown to be irregular cellular patterns and malignant cells were characterized by diverse characteristics.From small to medium-sized lymphocytes to large B cells, these cells of different morphology show the tumor characteristics of Lymphoma.And when large B cells begin to form significant flake structures, this suggests that the transition to more aggressive lymphomas such as diffuse large B cell lymphomas.This transformation occurs within 4-5 years of diagnosis in about 18% of patients.
Subtype, diagnosis and treatment
External marginal lymphoma has many subtypes, mainly divided according to the organs involved.These subtypes have an impact on the selection of treatment options through their correlation with infectious pathogens.
Basic MALT lymphoma, such as primary gastric MALT lymphoma, is usually a relatively benign disease, but the possibility of potential comorbidities and complications that accompany it cannot be ignored.
Taking primary gastric MALT lymphoma as an example, most cases of this disease are related to Helicobacter pylori infection, and more than 80% of patients demonstrate the presence of the pathogen.Treatment options generally include the use of antibiotics in combination with other therapies, so the prognosis of such lymphomas is usually good, with an overall 5-year survival rate ranging from about 86% to 95%.
Conclusion
External marginal zone lymphoma is a relatively diverse tumor that may exhibit different pathological characteristics in the human body with the presence of inflammation, infection and autoimmune response.The treatment and prognosis of these lymphomas also depend on the subtype of the lymphoma and its evolution process.For medical professionals who care for patients, how to accurately understand the formation process of MZL and its interrelationship with immune responses may provide more effective strategies for future clinical treatments.What other factors do you think can promote the development of these lymphomas in other organs?
