Molecular pathogenesis of mantle cell lymphoma
Pedro Jares, Dolors Colomer, Elias Campo
Pedro Jares, Dolors Colomer, Elias Campo
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Review Series

Molecular pathogenesis of mantle cell lymphoma

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.

Authors

Pedro Jares, Dolors Colomer, Elias Campo

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Figure 3

Multistep model in the progression of MCL.

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Multistep model in the progression of MCL. Clonal cells carrying the t(1...
Clonal cells carrying the t(11;14) translocation may be detected in the peripheral blood of healthy individuals at very low levels. The risk of progression of these clones must be extremely low, if any. Cells expressing cyclin D1 and carrying the t(11;14) may be found in the mantle zone of lymphoid follicles in otherwise reactive tissues. Most of these lesions will not evolve into an overt lymphoma. The incidental detection of tumor cells in the mantle zone of reactive tissues in patients with MCL who appear to be in complete remission suggests that this microenvironment may sustain chemoresistant cells. Anecdotal clinical observations provide a timeframe for the potential evolution of these lesions. Original magnification, ×100 (right and left), ×400 (center).