Abstract
Mantle Cell lymphoma (MCL) is a mature B-cell lymphoma with a well-known hallmark genetic alteration in most cases, t (11,14)(q13q32)/CCND1-IGH. However, our understanding of the genetic and epigenetic alterations in MCL has evolved over the years, and it is now known that translocations involving CCND2, or cryptic insertion of enhancer elements of IGK or IGL gene, can also lead to MCL. On a molecular level, MCL can be broadly classified into two subtypes, conventional MCL (cMCL) and non-nodal MCL (nnMCL), each with different postulated tumor cell origin, clinical presentation and behavior, mutational pattern as well as genomic complexity. This article reviews both the common and rare alterations in MCL on a gene mutational, chromosomal arm, and epigenetic level, in the context of their contribution to the lymphomagenesis and disease evolution in MCL. This article also summarizes the important prognostic factors, molecular diagnostic tools, and treatment options based on the most recent MCL literature.
Highlights
Mantle cell lymphoma (MCL) is a relatively uncommon subtype of mature B-cell lymphoma with a heterogenous tumor behavior, with most behaving aggressively while others following an indolent clinical course
The less common non-nodal variant, on the other hand, is derived from post-germinal center/memory-like B cells that are generally characterized by mutated immunoglobulin heavy chain variable region (IGHV), lack of SOX11 expression, low genomic complexity, and an indolent clinical behavior that is partially related to the lack of Genetics of Mantle Cell Lymphomas
This review focuses on the recent developments in the understanding of the mutational, methylation, and chromosomal-level alterations seen in MCL and the currently available molecular diagnostic tools and therapy options
Summary
Mantle cell lymphoma (MCL) is a relatively uncommon subtype of mature B-cell lymphoma with a heterogenous tumor behavior, with most behaving aggressively while others following an indolent clinical course. Based on the proposed model of molecular pathogenesis, two subtypes of MCL have been recognized, which differ in their clinical and biologic behavior [1] (Table 1). The more common conventional MCL (cMCL) arises from expansion of pre-germinal center/naïve-like B cells that are characterized by frequent expression of the transcription factor SOX11, higher likelihood of unmutated immunoglobulin heavy chain variable region (IGHV), high genomic complexity, and an aggressive clinical behavior. The less common non-nodal (leukemic) variant (nnMCL), on the other hand, is derived from post-germinal center/memory-like B cells that are generally characterized by mutated IGHV, lack of SOX11 expression, low genomic complexity, and an indolent clinical behavior that is partially related to the lack of
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