title: Familial Lymphoproliferative Disorders with Chromosomal Fragile Site Analysis.
abstract: We have identified a family in which three members developed B-cell lymphoproliferative disorders within a nine month period. The 33 year old proband and his mother have hairy cell leukemia, and his 37 year old brother developed a large cell lymphoma. Chromosomal fragile site analysis of peripheral blood lymphocytes of the three patients as well as two healthy family members was performed. The mean number of rare fragile sites present per cell analyzed was not significantly different from that observed in a group of healthy adults used as controls. However, the level of expression of the common fragile sites detected in each of the study patients was significantly elevated compared to the control population. Although the relationship between the level of expression of common fragile sites and the subsequent development of a malignant process is unknown, many of the agents that induce these sites are known mutagens, and the level of their expression may reflect a genetic susceptibility to mutagenic damage. Thus, it is possible that common exposure to an environmental mutagen may have contributed to the temporal clustering of malignancy in this family.
authors.affiliation: a Section of Hematology/Oncology, University of Chicago Medical Center, Chicago, Illinois, 60637, USA.
authors.forename: J A
authors.initials: JA
authors.lastname: Moormeier
authors.affiliation: a Section of Hematology/Oncology, University of Chicago Medical Center, Chicago, Illinois, 60637, USA.
authors.forename: M E
authors.initials: ME
authors.lastname: Neilly
authors.affiliation: b Department of Pathology, University of Chicago Medical Center, Chicago, Illinois, 60637, USA.
authors.forename: J W
authors.initials: JW
authors.lastname: Vardiman
authors.affiliation: a Section of Hematology/Oncology, University of Chicago Medical Center, Chicago, Illinois, 60637, USA.|b Department of Pathology, University of Chicago Medical Center, Chicago, Illinois, 60637, USA.
authors.forename: M M
authors.initials: MM
authors.lastname: Le Beau
authors.affiliation: a Section of Hematology/Oncology, University of Chicago Medical Center, Chicago, Illinois, 60637, USA.
authors.forename: H M
authors.initials: HM
authors.lastname: Golomb
keywords: Chromosomal fragile sites; Familial lymphoid neoplasms; Hairy cell leukemia; Lymphoproliferative disorders; Non-Hodgkin's lymphoma