| Literature DB >> 26834852 |
Maria A Loizidou1, Andreas Hadjisavvas2, George A Tanteles3, Elena Spanou-Aristidou3, Kyriacos Kyriacou2, Violetta Christophidou-Anastasiadou4.
Abstract
Fanconi anemia (FA) is a rare disorder characterized by multiple congenital malformations, progressive bone marrow failure and susceptibility to malignancies. Biallelic mutations in the breast cancer 2, early onset (BRCA2) gene are responsible for the FA-D1 subgroup, which accounts for ~3% of all the FA cases. Patients with biallelic BRCA2 mutations generally display a more severe phenotype, with earlier onset and increased incidence of leukaemia and other solid tumors, than other patients with FA. In the present report, the first Cypriot patient with FA-D1 is described, which is the fifth case of a homozygote for the same null allele reported thus far, and the third known case of neuroblastoma in association with FA-D1.Entities:
Keywords: Cyprus; Fanconi anemia; biallelic BRCA2 mutation; genotype-phenotype correlation; neuroblastoma
Year: 2015 PMID: 26834852 PMCID: PMC4727152 DOI: 10.3892/ol.2015.3852
Source DB: PubMed Journal: Oncol Lett ISSN: 1792-1074 Impact factor: 2.967