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]


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

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.[6]
  • 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.[7]
  • ISMCN is often found incidentally, occasionally in association with other types of lymphomas.


  1. 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. 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.
  3. Jaffe ES, Harris NL, Stein H, Isaacson PG (December 2008). "Classification of lymphoid neoplasms: the microscope as a tool for disease discovery". Blood. 112 (12): 4384–99. doi:10.1182/blood-2008-07-077982. PMC 2954680. PMID 19029456.
  4. Fisher RI (1996). "Mantle-cell lymphoma: classification and therapeutic implications". Ann. Oncol. 7 Suppl 6: S35–9. PMID 9010577.
  5. Pileri SA, Falini B (November 2009). "Mantle cell lymphoma". Haematologica. 94 (11): 1488–92. doi:10.3324/haematol.2009.013359. PMC 2770958. PMID 19880776.
  6. 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.
  7. 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.


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