Mantle cell lymphoma classification
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According to the revised 2016 World Health Organization classification of lymphoid neoplasms, mantle cell lymphoma (MCL) can be broadly classified into two types include classical MCL and leukemic nonnodal MCL. In-situ mantle cell neoplasia (ISMCN) is considered a separate entity, often as a precursor lesion, to the mantle cell lymphoma.
- Classical MCL typically develops from immunoglobulin heavy-chain variable region gene(IGHV)-unmutated or minimally mutated B cells that usually express SOX11 (SRY-Box 11 gene).
- Classical MCL usually involves the lymph nodes and other extranodal sites.
- However, these cells can acquire further molecular and cytogenetic abnormalities leading to the more aggressive:
Leukemic nonnodal MCL
- Leukemic nonnodal MCL typically develops from immunoglobulin heavy-chain variable region gene(IGHV)-mutated B cells that do not express SOX11 (SRY-Box 11 gene).
- Leukemic nonnodal MCL involve the peripheral blood (PB), bone marrow and the spleen.
- Although these are commonly clinically indolent in nature further mutations ,especially of TP53, can lead to more aggressive disease.
In-situ mantle cell neoplasia (ISMCN)
- It is characterized by the presence of cyclin D1 positive cells mostly in the inner mantle zones of follicles in lymphoid tissues that do not otherwise suggest the diagnosis of a MCL.
- The in situ lesion stage may be a common step in both SOX11-negative and positive subtypes of MCL as some in situ lesions express SOX11, whereas others are SOX11 negative.
- ISMCN is often found incidentally, occasionally in association with other types of lymphomas.
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