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What is mantle cell lymphoma?

Mantle cell lymphoma (MCL) is a relatively rare, unique subcategory of B-cell non-Hodgkin lymphoma (NHL) with a generally aggressive clinical course.[1]

Originally, MCL was classified with other types of lymphomas using different histology-based classification systems.[2] MCL was only classified as a distinct subtype of B-cell NHL in the 1994 Revised European-American Lymphoma Classification (REAL). It was then confirmed in the World Health Organization (WHO) classification system.[3]

MCL develops from changes to the outer edge, or mantle, of lymph nodes, causing the development of abnormal B-cells from that region.[4] It can also affect the spleen, blood and bone marrow.[4] MCL encompasses a wide range of biological and clinical variants.[5] The majority of patients present with advanced stage disease and require systemic therapy.[5]

Genomic instability & stability

A proposed model of molecular pathogenesis and progression of MCL.[6]
Adapted from Dreyling et al. 2017.

Statistics surrounding MCL

of non-Hodgkin lymphomas[1]
Median age at diagnosis[7]
3x more common in men[7]


MCL incidence rates

  • MCL has an annual incidence of one case per 200,000 people[7]
  • MCL represents approximately 5–7% of all malignant lymphomas in Western Europe[6]
  • MCL is typically sporadic, but it may have a higher incidence in some families[7]

Risk factors for MCL

The causes of MCL are mostly unknown; however, there are certain risk factors that are associated with the disease. These include:[8]

  • infection with certain viruses or bacteria
  • a weakened immune system
  • autoimmune disease
  • a previous cancer
  • having a close blood relative with MCL

MCL immunophenotype

MCL is a well-characterised B-cell lymphoma with markers readily detectable by immunohistochemistry[9]

  • t(11;14)(q13;q32) translocation associated with overexpression of cyclin D1. Cyclin D1 overexpression is a key event in MCL pathogenesis[1]
  • Overexpression of SOX-11 is observed in a majority of patients with MCL[1]

The majority of MCL cases also show expression of[10]:

  • CD20
  • CD5
  • BCL2

The more aggressive MCL subtypes, such as blastoid variants, display features such as[1][4]:

  • High Ki-67 proliferation index
  • p53 mutations and p16 deletions
Learn about the steps involved in diagnosing your patients’ MCL

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A dedicated online resource for healthcare professionals to learn about the diagnosis and management of rare hematological malignancies: Waldenström's macroglobulinemia and mantle cell lymphoma.

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ITEM CODE: CP-233134 | DATE OF PREPARATION: June 2021

References

Jain P, Wang M. Mantle cell lymphoma: 2019 update on the diagnosis, pathogenesis, prognostication, and management. Am J Hematol. 2019;94:710–725.
Harris N, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84(5):1361–1392.
Campo E, Pileri SA. The classification of lymphoma: updating the WHO classification. In: Hoffbrand A, et al, eds. Postgraduate Haematology. Wiley-Blackwell; 2011:614–638.
Vose J. Mantle cell lymphoma: 2017 update on diagnosis, risk-stratification, and clinical management. Am J Hematol. 2017;92:806–813.
Kim L, et al. EHA Endorsement of ESMO clinical practice guidelines for newly diagnosed and relapsed mantle cell lymphoma. HemaSphere. 2020;4(5):464.
Dreyling M, et al. Newly diagnosed and relapsed mantle cell lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2017;28(S4):iv62–iv71.
Lynch DT, Acharya U. Mantle cell lymphoma. In: StatPearls (Internet). StatPearls Publishing; 2021 Jan. Updated 2020 August 12. Accessed May 2021. https://www.ncbi.nlm.nih.gov/books/NBK536985/
Macmillan Cancer Support. Cancer information and support. Causes and risk factors of non-Hodgkin Lymphoma (NHL). Accessed May 2021. https://www.macmillan.org.uk/cancer-information-and-support/lymphoma/causes-and-risk-factors-of-non-hodgkin-lymphoma
Aqil B, et al. Immunophenotypic variations in mantle cell lymphoma and their impact on clinical behavior and outcome. Arch Pathol Lab Med. 2018;142(10):1268–1274.
McKay P, et al. A British Society for haematology good practice paper on the diagnosis and investigation of patients with mantle cell lymphoma. Br J Haematol. 2018;182:63–70.