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).[1][2]
- 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).[1][2]
- 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.[3]
- 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.[4]
- ISMCN is often found incidentally, occasionally in association with other types of lymphomas.
References
- ↑ 1.0 1.1 Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R; et al. (2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
- ↑ 2.0 2.1 Jares P, Colomer D, Campo E (2012). "Molecular pathogenesis of mantle cell lymphoma". J Clin Invest. 122 (10): 3416–23. doi:10.1172/JCI61272. PMC 3461905. PMID 23023712.
- ↑ Carvajal-Cuenca A, Sua LF, Silva NM, Pittaluga S, Royo C, Song JY; et al. (2012). "In situ mantle cell lymphoma: clinical implications of an incidental finding with indolent clinical behavior". Haematologica. 97 (2): 270–8. doi:10.3324/haematol.2011.052621. PMC 3269489. PMID 22058203.
- ↑ Christian B, Zhao W, Hamadani M, Sotomayor EM, Navarro W, Devine SM; et al. (2010). "Mantle cell lymphoma 12 years after allogeneic bone marrow transplantation occurring simultaneously in recipient and donor". J Clin Oncol. 28 (31): e629–32. doi:10.1200/JCO.2010.29.8992. PMID 20733121.