Familial Aggregation of Lymphoplasmacytic Lymphoma/Waldenstrom Macroglobulinemia with Solid Tumors and Myeloid Malignancies

• Lymphoplasmacytic lymphoma (LPL)/Waldenstrom macroglobulinemia (WM) is a B-cell disorder resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphoplasmacytic cells. LPL/WM is a very rare disease, with an incidence rate of 3-4 cases per million people per year. Currently, the causes of LPL/WM are poorly understood; however, there are emerging data to support a role for immune-related factors in the pathogenesis of LPL/WM. In addition, data show that genetic factors are of importance in the etiology of LPL/WM. In this paper, we will review the current knowledge about familiality of LPL/WM and provide novel data on solid tumors and myeloid malignancies in first-degree relatives of LPL/WM patients. Copyright (C) 2012 S. Karger AG, Basel

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Hematologi (hsv//swe)

MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Hematology (hsv//eng)

Keyword

Autoimmunity

Familial aggregation

Hematological malignancies

Lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia

Monoclonal

gammopathy of undetermined significance

Solid tumors

Susceptibility

Publication and Content Type

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