Familial aggregation of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia with solid tumors and myeloid malignancies.
Lymphoplasmacytic lymphoma (LPL)/Waldenström 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.
Division of Hematology, Department of Medicine, Karolinska University Hospital, Solna and Karolinska Institutet, Stockholm, Sweden. sigurdur.kristinsson@karolinska.se
Sigurdur Y
SY
Kristinsson
Lynn R
LR
Goldin
Ingemar
I
Turesson
Magnus
M
Björkholm
Ola
O
Landgren
D001327:Autoimmune Diseases / Q000473:pathology; D005192:Family Health; D006801:Humans; D007951:Leukemia, Myeloid / Q000150:complications* / Q000473:pathology*; D009369:Neoplasms / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D012307:Risk Factors; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000235:genetics / Q000473:pathology*