Abstract:
:Familial and metachronous aggregations of malignant lymphoma are well-documented, but the molecular basis of a predisposition for development of lymphoma is as yet unclear. Malignant lymphomas have been described as part of the spectrum of neoplasias in Li-Fraumeni syndrome (LFS), which is associated with constitutional mutations of p53. However, p53 germline mutations have also, albeit less frequently, been described in patients not fitting the clinical definition of LFS. To clarify whether a genetic predisposition for lymphoma is associated with constitutional p53 mutations, DNA from normal blood lymphocytes of 12 lymphoma patients with a family history of lymphoma and/or with metachronous lymphoma (median age 37 years) was examined for mutations of p53 exons 4-8. One patient had four first-degree relatives with Hodgkin's disease, acute leukemia, and carcinomas, but the family history did not fulfill criteria of LFS. Four patients with Hodgkin's disease were diagnosed with metachronous non-Hodgkin's lymphoma as a second malignant neoplasm. No constitutional p53 mutations were detected in any of these patients, implying that outside the clinical spectrum of LFS, constitutional p53 mutations are rare in patients with lymphomas.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Pötzsch C,Schaefer HE,Lübbert Mdoi
10.1002/(sici)1096-8652(199911)62:3<144::aid-ajh3>keywords:
subject
Has Abstractpub_date
1999-11-01 00:00:00pages
144-9issue
3eissn
0361-8609issn
1096-8652pii
10.1002/(SICI)1096-8652(199911)62:3<144::AID-AJH3>journal_volume
62pub_type
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