Mantle cell lymphoma
I See mantle cell lymphoma
Welcome to I See mantle cell lymphoma, a dedicated resource to help healthcare practitioners find the latest information about the signs, symptoms and diagnosis of MCL.
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]
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.
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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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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