Waldenström macroglobulinemia – immunophenotype and natural history
View Full Text  View/Add Comment  Download reader
KeyWord:Waldenstr?m macroglobulinemia, lymphoplasmacytic lymphoma, mature IgM+ B-lymphocyte, serum paraprotein, genetic predisposition, autoantibody syndrome
Siegfried Janz Department of Pathology, University of Iowa Carver College of Medicine, 1030ML, Iowa City, IA 52242 , USA.
Hits: 435
Download times: 605
      Despite encouraging progress in recent years, our knowledge of the natural history of Waldenstr?m macroglobulinemia (WM), a low-grade LPL (lymphoplasmacytic lymphoma) of mature IgM+ B-lymphocytes, remains superficial. This is particularly true of the etiology of WM (tumor causation and initiation) and the sequence of events that underlie the malignant transformation of precursor B cells (tumor progression). Here we briefly review the epidemiology of and genetic predisposition to WM and consider the role of autoimmunity and chronic inflammation in related tumor development. We discuss the immunophenotypic features of WM, including the immunological specificity of WM-associated IgM paraproteins. The proclivity of patients with WM to develop the rare immunoglobulin autoantibody syndromes mixed IgM-IgG cryoglobulinemia, chronic cold agglutinin disease, and IgM neuropathy will also be discussed. We conclude with a call for additional research to elucidate outstanding questions, such as the role of T cell-dependent vs. –independent immune responses in the pathophysiology of WM.