Mantle Cell Lymphoma
Overview
Plain-Language Overview
Mantle Cell Lymphoma is a type of cancer that affects the lymphatic system, which is part of the body's immune defense. It originates in a specific area of lymph nodes called the mantle zone, where certain white blood cells called B lymphocytes grow uncontrollably. This condition can cause swollen lymph nodes, fatigue, and sometimes problems with organs like the spleen or bone marrow. Because it involves the immune system, it can weaken the body's ability to fight infections. The disease often progresses over time and requires medical evaluation to determine the best approach to care.
Clinical Definition
Mantle Cell Lymphoma is a distinct subtype of non-Hodgkin B-cell lymphoma characterized by malignant proliferation of naive B cells originating from the mantle zone of lymphoid follicles. It is driven primarily by the chromosomal translocation t(11;14)(q13;q32), which leads to overexpression of the cyclin D1 protein, promoting cell cycle progression. This lymphoma typically presents with generalized lymphadenopathy, splenomegaly, and frequent bone marrow involvement. It is considered an aggressive lymphoma with a median survival of a few years despite treatment. Diagnosis relies on histopathology, immunophenotyping showing CD5 positivity and CD23 negativity, and detection of cyclin D1 overexpression. The disease often involves extranodal sites such as the gastrointestinal tract, manifesting as multiple lymphomatous polyposis.
Inciting Event
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Clinical Presentation
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Diagnostic Workup
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