Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Mantle Cell Lymphoma

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_3529-4

Synonyms

Definition

Mantle cell lymphoma is a B-cell neoplasia of monomorphic small- to medium-sized lymphoid cells with irregular nuclei, morphologically most resembling centrocytes.

Characteristics

Incidence

MCL comprises ~6 % of non-Hodgkin lymphoma.

Epidemiology

The majority of patients (2/3) are male, and the median age at diagnosis is 70 years. The etiology is unknown.

Pathogenesis

The causal molecular lesion is a reciprocal translocation t(11;14)(q13;q32), translocating the CCND1gene from chromosome 11 to chromosome 14, where it is brought under the control of the promoter of the constitutionally activated immunoglobulin heavy-chain (IgH) gene. Therefore, overexpression of cyclin D1 is present in virtually all cases of MCL. Most MCL cases also overexpress SOX11, a B-cell transcription factor. In practice, SOX-11 allows...

Keywords

Mantle Cell Lymphoma Total Body Irradiation Bone Marrow Involvement Lymphoblastic Lymphoma CCND1 Gene 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Department of HematologyThe Finsen Centre, RigshospitaletCopenhagenDenmark