Waldenström's Macroglobulinemia and Autoimmune Disease in a Family

  1. WILLIAM A. BLATTNER, M.D.;
  2. JUDY E. GARBER, B.S.;
  3. DEAN L. MANN, M.D.;
  4. ELISABETH A. McKEEN, M.D.;
  5. ROBIN HENSON, B.S.;
  6. DEBORAH B. McGUIRE, B.S.N.;
  7. WILLIAM B. FISHER, M.D.;
  8. ARTHUR W. BAUMAN, M.D.;
  9. LYNN R. GOLDIN, Ph.D.; and
  10. JOSEPH F. FRAUMENI, Jr., M.D.
  1. Bethesda, Maryland; Muncie, Indiana; and Rochester, New York

    Abstract

    We diagnosed Waldenstrom's macroglobulinemia in a father and three offspring. Clinical and subclinical autoimmune disorders occurred excessively in the family. The HLA haplotype A2, B8, DRw3 was detected in all patients with Waldenstrom's macroglobulinemia and all but one family member with autoimmune manifestations. A lod score [log odds] of 4.86 favors linkage to the HLA complex of a gene predisposing to lymphoproliferative and autoimmune disorders. Associated with this HLA haplotype were the B-cell alloantigens Ia-172 and 350, previously reported in patients with the lymphoma-prone sicca syndrome.

    Article and Author Information

    • ▸From the Environmental Epidemiology, Clinical Epidemiology, and Immunology Branches, National Cancer Institute, National Institutes of Health, Bethesda, Maryland; Ball Memorial Hospital, Muncie, Indiana; and Strong Memorial Hospital, Rochester, New York.

    • Grant support: in part by National Cancer Institute contracts to Litton-Bionetics (NO1-CP-61016) and Duke University Medical Center (NO1-CB-53890).

    • ▸Requests for reprints should be addressed to William A. Blattner, M.D.; Environmental Epidemiology Branch, National Cancer Institute, Landow Building, Room 3C-07; Bethesda, MD 20205.

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