Mantle cell lymphoma

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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