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Clin Lymphoma Myeloma Leuk. 2013 Apr;13(2):191-3. doi: 10.1016/j.clml.2013.02.017. Epub 2013 Mar 13.

Familial predisposition to monoclonal gammopathies: deviations in B-cell biology.

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Faculty of Medicine, University of Iceland, Reykjavík, Iceland.


Monoclonal gammopathies are associated with advancing age but a familial predisposition has been recognized for several decades. A functional phenotype, characterized by increased immunoglobulin (Ig) production after mitogen stimulation has been identified in healthy members of 4 families showing a predisposition toward IgM and IgG/IgA disorders. B cells from these hyperresponders do not show increased rates of Ig gene translocations and no aberrations were detected in an in vitro model of the germinal center reaction. Array-based comparative genome hybridization revealed deletions of Ig genes in peripheral blood B cells, as expected. In addition, random changes were detected throughout the genome, presumably reflecting off-target activation-induced cytidine deaminase (AID) activity. These random changes were significantly less prevalent in B cells from hyperresponders, indicating less exposure to the germinal center environment during maturation.

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