Risk factors for etiology and prognosis of mantle cell lymphoma

Abstract

Non-Hodgkin lymphomas (NHLs) include any kind of lymphoma except Hodgkin's lymphoma. Mantle cell lymphoma (MCL) is a B-cell NHL and it accounts for about 6% of all NHL cases. Its epidemiologic and clinical features, as well as biomarkers, can differ from those of other NHL subtypes. This article first provides a very brief description of MCL's epidemiology and clinical features. For etiology and prognosis separately, we review clinical, environmental, and molecular risk factors that have been suggested in the literature. Among a large number of potential risk factors, only a few have been independently validated, and their clinical utilization has been limited. More data need to be accumulated and effectively analyzed before clinically useful risk factors can be identified and used for prevention, diagnosis, prediction of prognosis path, and treatment selection.

Keywords::

• etiology
• mantle cell lymphoma
• prognosis
• risk factors

Acknowledgements

The authors thank the editor and three reviewers for their careful review and insightful comments, which have led to a significant improvement of this manuscript.

Financial & competing interests disclosure

This study has been supported by CA142774, CA165923 and CA182984 from NCI/NIH. The funder has no role in preparing and publishing the article. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Mantle cell lymphoma (MCL) is a rare, mostly aggressive subtype of non-Hodgkin lymphomas. Because of its low incidence rate, research on MCL remains limited.

• The epidemiology, etiology and prognostic patterns of MCL may differ from those of the other non-Hodgkin lymphoma subtypes.

• For etiology, a few lifestyle and occupational risk factors have been suggested, although not all have been confirmed. There is also evidence highlighting infectious agents and family history.

• In molecular studies, the most notable finding is CCND1. Other findings include SOX11, NOTCH1, ATM and others. There are conflicting observations in the literature. Additionally, miRNA, methylation and proteomic risk factors have also been suggested.

• For prognosis, the MCL International Prognostic Index has been developed using age, Eastern Cooperative Oncology Group performance status, lactate dehydrogenase and normalized white blood cell and has been validated in independent studies.

• Other possible prognostic factors include Ki-67, cell type, β-2 microglobulin and others.

• A large number of molecular risk factors have been suggested, including SOX11, SOCS3, the B-cell receptor signaling pathway and others. Epigenetic risk factors have also been suggested.

• There is a lack of consensus on risk factors. More carefully designed and controlled epidemiologic studies/clinical trials and more comprehensive profiling studies are needed.