Immunoglobulin Type M Monoclonal Gammopathy of Undetermined Significance (IgM-MGUS)

  • Mary L. McMaster
  • Helga M. Ögmundsdóttir
  • Sigurdur Y. Kristinsson
  • Robert A. Kyle


The term monoclonal gammopathy pertains to the expansion of a single clone of B lymphocytes that produce an excess of a homogeneous immunoglobulin. Monoclonal gammopathy of undetermined significance (MGUS) is diagnosed in a patient who presents with a monoclonal gammopathy in the absence of histologic evidence, signs, or symptoms of a malignant lymphoproliferative or plasmacytic disorder. MGUS is among the most common premalignant conditions in Western populations, having a prevalence of about 4 % in white adults older than age 50. The immunoglobulin type M (IgM) subtype of MGUS (IgM-MGUS) exhibits unique features that distinguish it from non-IgM MGUS, including a distinctive racial prevalence pattern and spectrum of malignant outcomes. Putative risk factors predisposing to development of IgM-MGUS have been identified, but most await confirmation. The MYD88 L265P mutation that is characteristic of Waldenström macroglobulinemia (WM) is found in about half of IgM-MGUS patients. The most common malignant outcome is WM, but patients can develop other lymphoproliferative B-cell diseases. Patients progress to WM at a rate of 1.5 % per year, and they continue to be at risk of progression more than 20 years following diagnosis. Size of the monoclonal component at diagnosis has been the most consistent predictor of prognosis. IgM MGUS is also associated with other nonmalignant comorbidities, and patients diagnosed with it have inferior survival compared to the general population. Thus, there is growing consensus that IgM monoclonal gammopathy may indeed have clinical significance. Further, while routine screening for IgM-MGUS is not widely advocated, it is recommended that, when discovered, IgM-MGUS patients should be followed for life.


Multiple Myeloma Chronic Lymphocytic Leukemia Monoclonal Gammopathy Undetermined Significance Class Switch Recombination 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  • Mary L. McMaster
    • 1
    • 2
  • Helga M. Ögmundsdóttir
    • 3
  • Sigurdur Y. Kristinsson
    • 3
    • 4
  • Robert A. Kyle
    • 5
  1. 1.Division of Cancer Epidemiology and Genetics, Department of Health and Human ServicesNational Cancer Institute, National Institutes of HealthBethesdaUSA
  2. 2.Commissioned Corps, Department of Health and Human ServicesUnited States Public Health ServiceWashington, DCUSA
  3. 3.Faculty of MedicineUniversity of IcelandReykjavikIceland
  4. 4.Department of MedicineKarolinska University Hospital and Karolinska InstituteStockholmSweden
  5. 5.Division of Hematology and internal MedicineMayo Clinic College of MedicineRochesterUSA

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