Autoimmunity and lymphoma: is mantle cell lymphoma a mistake of the receptor editing mechanism?

Abstract

Mantle cell lymphoma (MCL) is an aggressive B cell malignancy, which is believed to originate from naïve B cells in the mantle zone of lymph nodes. We speculate that a possible mechanistic hypothesis for the generation of MCL is one in which receptor editing and germinal centre exclusion could be involved in the molecular development and in the display of clinical characteristics of this rare, aggressive and scarcely understood lymphoma. The hypothesis is supported by a preferential autoimmune related IGVH gene utilization in MCL, where VH3-21, VH4-34 and VH5-51 genes are predominant, and by the fact that MCL expresses immunoglobulin light chain (IgL) lambda more frequently than other non-Hodgkin's lymphomas and that IgL lambda-producing B cells usually delete one or both their IgL kappa genes.