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Familial Aspects of Chronic Lymphocytic Leukemia, Monoclonal B-Cell Lymphocytosis (MBL), and Related Lymphomas

Author(s): Lynn R Goldin | Ola Landgren | Gerald E Marti | Neil E Caporaso

Journal: European Journal of Clinical & Medical Oncology
ISSN 1759-8958

Volume: 2;
Issue: 1;
Start page: 119;
Date: 2010;
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Keywords: chronic lymphocytic leukemia | monoclonal B-cell lymphocytosis | familial risk | germ line susceptibility | environmental risk factors

Families with multiple individuals affected with chronic lymphocytic leukemia (CLL) and other related B-cell tumors have been described in the literature. Familial CLL does not appear to differ from sporadic CLL in terms of prognostic markers and clinical outcome. Although some environmental factors (such as farming-related exposures and occupational chemicals) may increase the risk of CLL, results of epidemiological studies have been generally inconsistent, and well-defined extrinsic risk factors are unknown. Large, population-based, case–control and cohort studies have also shown significant familial aggregation of CLL and related conditions including non-Hodgkin lymphomas, especially other indolent lymphomas. The precursor condition, monoclonal B-cell lymphocytosis (MBL), also aggregates in CLL families. However, because the baseline population risks for CLL and other indolent lymphomas are low, the absolute risk to a first-degree relative of developing CLL or a related disease is also low. Linkage studies have been conducted in high-risk CLL families to screen the whole genome for loci that contribute to susceptibility, but no gene mutations have yet been identified by this method. Association studies of candidate genes have implicated several genes as being important in CLL, but more studies are needed to verify these findings. Results from whole-genome association are promising. The ability to conduct large-scale genomic studies will play an important role in detecting susceptibility genes for CLL over the next few years and thereby helping to delineate etiologic pathways.
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