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Blood, 15 July 2005, Vol. 106, No. 2, pp. 668-672.
Prepublished online as a Blood First Edition Paper on April 5, 2005; DOI 10.1182/blood-2005-01-0140.


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Submitted January 11, 2005
Accepted March 16, 2005

Familial risk of non-Hodgkin lymphoma and other lymphoproliferative malignancies by histopathologic subtype from the Swedish family-cancer database

Andrea Altieri*, Justo L Bermejo, and Kari Hemminki

Division of Molecular Genetic Epidemiology, German Cancer Research Centre, Heidelberg, Germany
Division of Molecular Genetic Epidemiology, German Cancer Research Centre, Heidelberg, Germany; Department of Biosciences at Novum, Karolinska Institute, Huddinge, Sweden

* Corresponding author; email: a.altieri{at}dkfz-heidelberg.de.

Non-Hodgkin lymphoma (NHL) consists of a heterogeneous group of tumors. Population-based data on the familial risk of specific histopathologic subtypes have not been established. Such data are useful for clinical counseling and searching tumor subtypes sharing common genetic pathways. We used the Swedish Family-Cancer Database to calculate standardized incidence ratios (SIRs) for histopathologic-specific subtypes of NHL in 4,455 offspring with NHL with parents or siblings affected with different types of lymphoproliferative malignancies. A familial history of NHL increased significantly the risk of NHL (SIRparent=1.8, SIRsibling=1.9), diffuse large B-cell (SIRparent=2.3), follicular (SIRsibling=2.3) and B-cell-NOS (SIRsibling=3.4). For a parental history of histopathologic-specific concordant cancer the risks were significantly increased for diffuse large B-cell (SIR=11.8), follicular NHL (SIR=6.1), plasma cell myeloma (SIR=2.5) and chronic lymphocytic leukemia (SIR=5.9). The familial clusters for NHL seemed stronger in females and in siblings. Our study provides the first quantification of the familial risks for NHL by histopathology. The present findings give evidence for a strong familial association of NHL, with little differences in the magnitude of risks for various histopathologic subtypes. The patterns of risks in parents and siblings support the hypothesis of an autosomal dominant component for diffuse large B-cell NHL and a recessive one for follicular NHL.


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