Mantle cell lymphoma classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ali Akram, M.B.B.S.[2]
Overview
According to the revised 2016 World Health Organization classification of lymphoid neoplasms, mantle cell lymphoma(MCL) can be broadly classified into three types:
- Classical MCL.
- Leukemic nonnodal MCL.
- In-situ mantle cell neoplasia (ISMCN).
Classification
Classical MCL:
- 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:
- Blastoid MCL
- Pleomorphic MCL
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+ 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.