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.
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
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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.
This site has been developed by Janssen-Cilag International NV. Janssen-Cilag International NV is the responsible editor of this document.
ITEM CODE: CP-233134 | DATE OF PREPARATION: June 2021
