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Blood, 15 September 2004, Vol. 104, No. 6, pp. 1850-1854.
Prepublished online as a Blood First Edition Paper on May 25, 2004; DOI 10.1182/blood-2004-01-0341.


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Submitted January 28, 2004
Accepted May 10, 2004

Familial Risk of Lymphoproliferative Tumors in Families of Patients with Chronic Lymphocytic Leukemia: Results from the Swedish Family-Cancer Database

Lynn R Goldin*, Ruth M Pfeiffer, Xinjun Li, and Kari Hemminki

Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD, USA
Biostatistics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD, USA
Dept. of Biosciences at Novum, Karolinska Institute, Stockholm, Sweden
Dept. of Biosciences at Novum, Karolinska Institute, Stockholm, Sweden; Division of Molecular Genetic Epidemiology, German Cancer Research Center, Heidelberg, Germany

* Corresponding author; email: goldinl{at}mail.nih.gov.

The importance of genetic factors in etiology of chronic lymphocytic leukemia (CLL) is suggested by family and population studies. However, the spectrum of malignancies sharing common genetic factors with CLL and the effects of gender and age on familial risk are unknown. We used the Swedish Family Cancer Database to test for increased familial risks of CLL and other lymphoproliferative tumors. Cancer diagnoses from 1958-1998 were assessed in 14,336 first-degree relatives of 5,918 CLL cases and in 28,876 first-degree relatives of 11,778 controls. Cancer risks in relatives of cases were compared to those in relatives of controls using marginal survival models. Relatives of cases were at significantly increased risk for CLL with a relative risk (RR) of 7.52 (95% CI:3.63-15.56), for non-Hodgkin lymphoma (RR=1.45, 95% CI:0.98-2.16) and for Hodgkin lymphoma (RR=2.35, 95% CI:1.08-5.08). CLL risks were similar in parents, siblings, and offspring of cases, in male and female relatives, and were not affected by the cases' age at diagnosis. Anticipation was not significant when analyzed using lifetable methods. We conclude that the familial component for CLL is shared with other lymphoproliferative malignances suggesting common genetic pathways. However, because clinically diagnosed CLL is uncommon, absolute excess risk to relatives is small.


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