Mantle cell lymphoma

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Mantle Cell Lymphoma

Background

From wikipedia:Mantle_Cell_Lymphoma

Mantle cell lymphoma (MCL) is one of the rarer of the non-Hodgkin's lymphomas (NHLs), comprising about 6% of NHL cases.[1] There are only about 15,000 patients presently in the U.S. (The prevalence seems to be somewhat higher in Europe.) While it is difficult to treat and seldom considered cured, investigations into better treatments are actively pursued worldwide. Median survival times were about 3 years, but are now estimated as approaching 6 years for new patients.

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).[3][4]

The cause is unknown and not genetic. MCL is not communicable. It essentially is an abnormal break and subsequent translocation in a gene that causes the cells to divide too early before becoming capable of helping to fight diseases. In addition, the cells do not die as they should and therefore accumulate in the lymphoid system, including lymph nodes and the spleen, with non-useful cells eventually rendering the system dysfunctional. MCL affected cells proliferate in a nodular or diffuse pattern with two main cytologic variants: typical or blastic. Typical cases are small to intermediate sized cells with irregular nuclei. Blastic (aka blastoid) variants have intermediate to large sized cells with finely dispersed chromatin and are more aggressive in nature.[5]

Current Treatments

Bortezomib

Further Reading

wikipedia:Mantle_Cell_Lymphoma

Lymphomation.org

Mantle Cell Lymphoma Consortium