Mantle cell lymphoma

Mantle cell lymphoma is a B-cell lymphoma thought to be the malignant counterpart of the memory B cells found in the mantle zone of lymphoid follicles. It has characteristic cell surface markings of CD5+, CD10-, CD23-. Clinically, it is characterized by a moderate rate of growth. While it often responds to cytotoxic chemotherapy, it has frustrated attempts at cure with chemotherapy, though there are reports of long-term survivors following allogeneic bone marrow transplantation. The median survival is generally 3-5 years.

Mantle cell lymphoma is almost uniformly characterized, using classical cytogenetics or PCR, by a t(11;14) which puts the cyclin D1 (also known as BCL-1, or B-cell leukemia/lym-phoma 1) gene under control of the Ig heavy chain transcription control elements. Cyclin D1 binds to and activates cyclin-dependent kinases. An important target of this activated cyclin-dependent kinase complex is the retinoblastoma (RB) gene product. In its hypophosphorylated state, RB inhibits entry into S-phase of the cell cycle by binding the transcription factor E2F. When RB is phosphorylated, E2F is freed to activate the transcription of genes which propel the cell into S-phase. Therefore, overexpression of cyclin D1 acts to overcome this late Gl-phase checkpoint and maintain continuous proliferation.

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