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Familial Waldenstrom's macroglobulinemia.

Mary L McMaster1

  • 1Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, MD, USA.

Seminars in Oncology
|April 30, 2003
PubMed
Summary

Familial Waldenstrom

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Area of Science:

  • Hematology
  • Oncology
  • Genetics

Background:

  • The exact cause of Waldenstrom's macroglobulinemia (WM) remains unknown.
  • Reports of familial clustering suggest a potential genetic component in WM etiology.
  • The proportion of WM cases occurring in a familial setting is not well-defined.

Purpose of the Study:

  • To review existing data on familial Waldenstrom's macroglobulinemia.
  • To investigate potential differences between familial and sporadic WM.
  • To explore the prevalence of immunologic abnormalities in relatives of WM patients.

Main Methods:

  • Review of published data on 12 families with Waldenstrom's macroglobulinemia since 1962.
  • Analysis of immunologic abnormalities in first-degree relatives of WM cases.
  • Comparison of IgM monoclonal gammopathy (IgM MG) prevalence in relatives versus general population.

Main Results:

  • Familial WM may present distinct characteristics compared to sporadic cases.
  • A significant occurrence of various immunologic abnormalities was observed in WM relatives.
  • Prevalence of IgM MG in first-degree relatives was 6.3%, a tenfold increase over general population estimates.

Conclusions:

  • Familial clustering and specific immunologic abnormalities suggest a genetic predisposition to WM.
  • The high prevalence of IgM MG in relatives warrants further investigation due to its known progression to WM.
  • Despite limitations, findings support systematic studies of WM within families.

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