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Concept
Mantle cell lymphoma
Summary
Mantle cell lymphoma (MCL) is a type of non-Hodgkin's lymphoma (NHL), comprising about 6% of NHL cases. It is named for the mantle zone of the lymph nodes.
MCL is a subtype of B-cell lymphoma, due to CD5 positive antigen-naive pregerminal center B-cell within the mantle zone that surrounds normal germinal center follicles. MCL cells generally over-express cyclin D1 due to the t(11:14) translocation, a chromosomal translocation in the DNA.
People with mantle cell lymphoma typically present with symptoms later in life, with a median age of onset between 60 and 70 years of age. People commonly present with a non-localizing lymphadenopathy (enlarged lymph nodes) with B symptoms including fevers, chills and night sweats sometimes being present. 80% of patients present with stage 3 or 4 disease (advanced disease) at the time of diagnosis, with involvement of the bone marrow, liver or gastrointestinal tract. 25% of patients present with a bulky lymphadenopathy characterized by lymph nodes greater than 10 cm in size. Other patients may present with central nervous system involvement, which is associated with a very poor prognosis.
A rare subtype, known as non-nodal mantle cell lymphoma, presents without lymph node swelling (non-nodal) with circulating lymphoma cells (leukemic presentation). This type of mantle cell lymphoma is associated with a more indolent, asymptomatic and slowly progressive course, however malignant transformation to aggressive forms is possible.
Mantle cell lymphoma has been reported in rare cases to be associated with severe allergic reactions to mosquito bites. These reactions involve extensive allergic reactions to mosquito bites which range from greatly enlarged bite sites that may be painful and involve necrosis to systemic symptoms (e.g. fever, swollen lymph nodes, abdominal pain, and diarrhea), or, in extremely rare cases, to life-threatening anaphylaxis. In several of these cases, the mosquito bite allergy (MBA) reaction occurred prior to the diagnosis of MCL suggesting that MBA can be a manifestation of early-developing mantle cell lymphoma.
Official source
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