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* Classical MCL usually involves the [[lymph nodes]] and other extranodal sites. | * 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: | * 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: === | ||
Revision as of 18:16, 2 January 2019
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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 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.
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.