title: Clinical characteristics of familial B-CLL in the National Cancer Institute Familial Registry.
abstract: In an ongoing study, families with two or more living cases of B-CLL in first-degree relatives have been recruited through physician and self-referral. Since 1967, 28 kindreds with 73 cases of B-CLL have been enrolled within the National Cancer Institute (NCI) Familial B-CLL Registry. Medical, clinical, and demographic information have been obtained from private physicians, patient interview, hospital records, and death certificates. We used SEER Registry data to compare characteristics of sporadic B-CLL to familial B-CLL. The mean age at diagnosis was approximately 10 years younger among familial cases (57.9 +/- 12.1) than that observed in sporadic cases (70.1 +/- 11.9). A higher percentage of second primary tumors among familial CLL cases compared to reports in sporadic was also observed (16% vs. 8.8%). However, the transformation rate to non-Hodgkin's lymphoma does not appear to be different from that reported for sporadic cases. In conclusion, we observed some differences between familial and sporadic cases; whether any of these characteristics affect survival time or severity of disease is unknown. The study of families with multiple B-CLL cases will aid in delineating the genes and environmental factors that may play a role in the development of both forms of B-CLL.
authors.affiliation: Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, 6120 Executive Blvd., MSC 7236, Rockville, MD 20852, USA.
authors.forename: N
authors.initials: N
authors.lastname: Ishibe
authors.forename: M T
authors.initials: MT
authors.lastname: Sgambati
authors.forename: L
authors.initials: L
authors.lastname: Fontaine
authors.forename: L R
authors.initials: LR
authors.lastname: Goldin
authors.forename: N
authors.initials: N
authors.lastname: Jain
authors.forename: N
authors.initials: N
authors.lastname: Weissman
authors.forename: G E
authors.initials: GE
authors.lastname: Marti
authors.forename: N E
authors.initials: NE
authors.lastname: Caporaso
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D002471:Cell Transformation, Neoplastic; D005192:Family Health; D005260:Female; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000453:epidemiology / Q000235:genetics*; D008137:Longitudinal Studies; D008228:Lymphoma, Non-Hodgkin; D008297:Male; D008875:Middle Aged; D009316:National Institutes of Health (U.S.); D016609:Neoplasms, Second Primary; D010375:Pedigree; D012042:Registries / Q000706:statistics & numerical data*; D012075:Remission, Spontaneous; D014481:United States