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mantle cell lymphoma

Mantle cell lymphoma is one of the rarest of the non-Hodgkin's lymphomas (NHLs), comprising about 6% of NHL cases. 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 a t(11:14)[2] chromosomal translocation in the DNA. More specifically, the translocation is at t(11;14)(q13;q32).

There are no proven standards of treatment for MCL, and not even consensus among specialists on how to treat it optimally. Many regimens are available and often get good response rates, but patients almost always get disease progression after chemotherapy. Each relapse is typically more difficult to treat, and relapse is generally faster. Fortunately, regimens are available that will treat relapse, and new approaches are under test. Because of the aforementioned factors, many MCL patients enroll in clinical trials to get the latest treatments. There are four classes of treatments currently in general use: chemotherapy, immune based therapy, radioimmunotherapy and new biologic agents. The phases of treatment are generally: frontline, following diagnosis, consolidation, after frontline response (to prolong remissions), and relapse. Relapse is usually experienced multiple times.

ABREVIATION(S):
MCL

TYPE: blood cell cancer

Related patway(s): DNA damage response (DDR)

DNAtraffic protein(s) related to disease: ATM

OMIM: xxx6

Last modification date: July 2, 2011