Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma (NHL) and accounts for ~5% of all NHL. It is a malignant neoplasm of virgin B cells that closely resemble normal mantle zone B cells surrounding germinal centers.
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Epidemiology
They occur in older adults (mean age ~60 years), and there is a recognized male predilection (M: F of ~4:1) 2.
MCL is usually widespread at diagnosis and apart from lymphadenopathy, frequently involves the spleen, bone marrow, and gastrointestinal tract.
Pathology
MCL is characterized by an infiltrate of small to medium-sized cells with folded nuclei and scant cytoplasm. The diagnosis of MCL is confirmed by histological assessment and immunohistochemical evaluation, including cyclin D1.
Genetics
Many cases have the t(11;14)(q13;q32) translocation that causes overexpression of cyclin-D1 2.
Staging
Staging is similar to standard non-Hodgkin lymphoma staging:
stage I: involvement of one lymph node or group of adjacent lymph nodes or one extra lymphatic site
stage II: involvement of two or more nodal groups on the same side of the diaphragm or stage I or II with contiguous extra-nodal involvement
stage III: involvement of lymph nodes on either side of the diaphragm
stage IV: involvement of bone marrow or non-contiguous extra-nodal involvement
See also: Lugano staging classification
Treatment and prognosis
Survival is short, with a ~50% 5-year survival. Over one-third of the patients can die within a year despite the administration of aggressive combination chemotherapy.