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Waldenström’s Macroglobulinemia pp 143–167Cite as

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

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Abstract

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.

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Authors and Affiliations

  1. Division of Cancer Epidemiology and Genetics, Department of Health and Human Services, National Cancer Institute, National Institutes of Health, 9609 Medical Center Drive, Room 6E516, MSC 9769, Bethesda, MD, 20892-9769, USA

    Mary L. McMaster

  2. Commissioned Corps, Department of Health and Human Services, United States Public Health Service, Washington, DC, USA

    Mary L. McMaster

  3. Faculty of Medicine, University of Iceland, Reykjavik, Iceland

    Helga M. Ögmundsdóttir & Sigurdur Y. Kristinsson

  4. Department of Medicine, Karolinska University Hospital and Karolinska Institute, Stockholm, Sweden

    Sigurdur Y. Kristinsson

  5. Division of Hematology and internal Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA

    Robert A. Kyle

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  1. Mary L. McMaster
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  2. Helga M. Ögmundsdóttir
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  3. Sigurdur Y. Kristinsson
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  4. Robert A. Kyle
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Correspondence to Mary L. McMaster .

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Editors and Affiliations

  1. Haematology, Hôpital Pitié-Salpêtriêre, Paris, France

    Véronique Leblond

  2. Dana Farber Cancer Institute, Boston, Massachusetts, USA

    Steve Treon

  3. School of Medicine, University of Athens, Athens, Greece

    Meletios Dimoploulos

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McMaster, M.L., Ögmundsdóttir, H.M., Kristinsson, S.Y., Kyle, R.A. (2017). Immunoglobulin Type M Monoclonal Gammopathy of Undetermined Significance (IgM-MGUS). In: Leblond, V., Treon, S., Dimoploulos, M. (eds) Waldenström’s Macroglobulinemia. Springer, Cham. https://doi.org/10.1007/978-3-319-22584-5_11

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