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由于BRCA2基因塞浦路斯奠基者突变纯合性导致的范可尼贫血-D1:一例报告。

Fanconi anemia-D1 due to homozygosity for the BRCA2 gene Cypriot founder mutation: A case report.

作者信息

Loizidou Maria A, Hadjisavvas Andreas, Tanteles George A, Spanou-Aristidou Elena, Kyriacou Kyriacos, Christophidou-Anastasiadou Violetta

机构信息

Department of Electron Microscopy/Molecular Pathology, Cyprus Institute of Neurology and Genetics, Nicosia 1683, Cyprus.

Department of Electron Microscopy/Molecular Pathology, Cyprus Institute of Neurology and Genetics, Nicosia 1683, Cyprus; Cyprus School of Molecular Medicine, Cyprus Institute of Neurology and Genetics, Nicosia 1683, Cyprus.

出版信息

Oncol Lett. 2016 Jan;11(1):471-473. doi: 10.3892/ol.2015.3852. Epub 2015 Nov 2.

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.

摘要

范可尼贫血(FA)是一种罕见的疾病,其特征为多种先天性畸形、进行性骨髓衰竭以及易患恶性肿瘤。乳腺癌2号基因(BRCA2)双等位基因突变导致了FA-D1亚组,该亚组约占所有FA病例的3%。与其他FA患者相比,携带BRCA2双等位基因突变的患者通常表现出更严重的表型,发病更早,白血病和其他实体瘤的发病率更高。在本报告中,描述了首例塞浦路斯FA-D1患者,这是迄今为止报道的同一无效等位基因纯合子的第五例病例,也是已知的第三例与FA-D1相关的神经母细胞瘤病例。

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