Petrucelli Nancie, Mange Sarah, Fulbright Jennifer L, Dohany Lindsay, Zakalik Dana, Duquette Debra
Cancer Genetic Counseling Service, Karmanos Cancer Institute, 4100 John R, HPO3GC, Detroit, MI, 48201, USA,
J Genet Couns. 2015 Apr;24(2):285-93. doi: 10.1007/s10897-014-9762-z. Epub 2014 Sep 9.
This study determined the prevalence of non-Ashkenazi Jewish BRCA1/2 mutations in the Ashkenazi Jewish population in the state of Michigan, current provider testing practices, and the use of mutation probability models in determining which Ashkenazi Jewish individuals should be offered further analysis following negative BRCA1/2 founder testing. Testing patterns, mutation probabilities, and testing results were assessed for 327 Ashkenazi Jewish individuals seen for BRCA1/2 counseling in the state of Michigan who underwent testing for the Ashkenazi Jewish founder mutations. Only one (0.6 %) Ashkenazi Jewish individual with sequencing after negative founder analysis was found to have a non-founder mutation; no rearrangements were identified. Testing patterns varied by clinic, with the proportion of Ashkenazi Jewish individuals undergoing additional sequencing ranging from 22.2 to 92.9 %. In Ashkenazi Jewish individuals with a pre-test BRCAPRO risk calculation, the mean risk was significantly higher in those with follow-up sequencing compared to those who did not pursue additional testing. The low prevalence of non-founder BRCA1/2 mutations in Ashkenazi Jewish individuals does not warrant automatically reflexing to full analysis after negative mutation testing. Increased use of mutation probability models may aid in determining which cases warrant additional testing.
本研究确定了密歇根州阿什肯纳兹犹太人群体中非阿什肯纳兹犹太BRCA1/2突变的患病率、当前医疗服务提供者的检测实践,以及在BRCA1/2始祖突变检测结果为阴性后,使用突变概率模型来确定哪些阿什肯纳兹犹太个体应接受进一步分析。对密歇根州327名因BRCA1/2咨询前来就诊并接受阿什肯纳兹犹太始祖突变检测的阿什肯纳兹犹太个体的检测模式、突变概率和检测结果进行了评估。在始祖分析结果为阴性后进行测序的阿什肯纳兹犹太个体中,仅发现1例(0.6%)有非始祖突变;未发现重排。检测模式因诊所而异,接受额外测序的阿什肯纳兹犹太个体比例在22.2%至92.9%之间。在检测前进行BRCAPRO风险计算的阿什肯纳兹犹太个体中,进行后续测序的个体的平均风险显著高于未进行额外检测的个体。阿什肯纳兹犹太个体中非始祖BRCA1/2突变的低患病率并不意味着在突变检测结果为阴性后就自动进行全面分析。更多地使用突变概率模型可能有助于确定哪些病例需要进行额外检测。