The cellular origin of mantle cell lymphoma

Mantle cell lymphoma accounts for 5–10% of all non-Hodgkin's lymphomas and it has one the worst prognosis among all lymphomas. There is no therapy that can be considered as standard. Mantle cell lymphoma can show different “architectural” patterns as well different morphologic variants. Mantle cell lymphoma is believed to derive from marginal zone or peripheral blood memory B-cells. The immunophenotype of the neoplastic cells reflect the phenotype of a mature B-cell, even if mantle cell lymphoma cells are typically CD5+ and CD23−. Mantle cell lymphoma is characterized by the deregulated expression of cyclins D, mainly of cyclin D1, which is targeted by the t(11;14)(q13;q32) chromosomal translocation, the genetic hallmark of the disease. In this review will summarize the main morphologic and immunophenotypic features of the neoplastic cells, and the genetics and biology underlying the disease.Cell facts
• Mantle cell lymphoma (MCL) is a lymphoid tumor believed to derive from marginal zone or peripheral blood memory B-cells.
• MCL is characterized by a deregulated expression of cyclins D, mainly of cyclin D1 which is constitutively expressed due to the juxtaposition of the gene to the immunoglobulin heavy chain genes promoter region in the t(11;14)(q13;q32) chromosomal translocation.
• MCL shows a heavy deregulation of the cell cycle and activation of PI3K/AKT, mTOR and NF-kB pathways, possibly offering therapeutic targets to improve patients’ outcome.
• MCL has a very poor prognosis.