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J Clin Invest. 2012 Oct;122(10):3416-23. doi: 10.1172/JCI61272. Epub 2012 Oct 1.

Molecular pathogenesis of mantle cell lymphoma.

Author information

1
Hematopathology Section, Department of Pathology, Hospital Clinic, Institut d’Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain.

Abstract

Mantle cell lymphoma is a B cell malignancy in which constitutive dysregulation of cyclin D1 and the cell cycle, disruption of DNA damage response pathways, and activation of cell survival mechanisms contribute to oncogenesis. A small number of tumors lack cyclin D1 overexpression, suggesting that its dysregulation is always not required for tumor initiation. Some cases have hypermutated IGHV and stable karyotypes, a predominant nonnodal disease, and an indolent clinical evolution, which suggests that they may correspond to distinct subtypes of the disease. In this review, we discuss the molecular pathways that contribute to pathogenesis, and how improved understanding of these molecular mechanisms offers new perspectives for the treatment of patients.

PMID:
23023712
PMCID:
PMC3461905
DOI:
10.1172/JCI61272
[Indexed for MEDLINE]
Free PMC Article

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