Mantle cell lymphoma is an uncommon variant of mature B cell lymphoma (< 10%) that usually behaves as an intermediate lymphoma (eg. similar to DLBCL); some cases are more indolent.
Basic Sciences
This lymphoma is usually comprised of small to medium sized lymphocytes with cleaved nuclei. The characteristic finding is positive staining for cyclin D1 in the nucleus, most often due to a translocation of the cyclin D1 gene with the IgH gene. The surface of the cell also stains positively for IgM and IgD. Like most mature B cell neoplasms, expression of CD20 is typical.
Diagnosis
Patients typically present with advanced disease; B symptoms are not uncommon (1/3rd of patients). Extranodal sites may be involved in one quarter of patients, often the gastrointestinal tract.
Examination should pick up enlarged lymph nodes.
Staging with PET/CT is usually appropriate. Prognostication is best achieved with the FLIPI score; 5 year survival ranges from 65% (low risk) to 8% (high risk).
Management
A small percentage of patients with indolent disease can be managed similarly to follicular lymphoma (observation, cytotoxic therapy for symptomatic progression).
The majority of patients present with advanced disease and need therapy to prolong survival. The mainstay of treatment is chemotherapy with rituximab, usually with R-Hyper-CVAD (dex instead of pred); autologous transplantation may have a role in young and fit patients but late relapse is common; 5 year survival after autologous HSCT is 50-60%.
For patients with truly localised mantle cell lymphoma, cure may be possible with involved field radiotherapy and systemic treatment, but this has not been evaluated in trials.
Given the lack of specific data, I would treat localised mantle cell lymphoma as per DLBCL.
