Mantle cell lymphoma

Specialties

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.


What are the features of MCL, its clinical course and its variants?

In this video you can learn more about the pathogenesis and the progression of MCL, its different subtypes and how to identify the disease.

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CP-299785, approved on 11/05/2022

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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