Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV003486520 | SCV004240797 | pathogenic | Hereditary factor VIII deficiency disease | 2023-12-11 | criteria provided, single submitter | clinical testing | Variant summary: The variant involves the deletion of exons 7-13 in the F8 gene. A presumed nomenclature of c.(787+1_788-1)_(2113+1_2114-1)del has been designated for the purposes of this classification. The variant was absent in 16079 control chromosomes (gnomAD, structural variants dataset). c.(787+1_788-1)_(2113+1_2114-1)del has been reported in the literature in individuals affected with Factor VIII Deficiency (Hemophilia A) (examples: Minno_2014, Pinto_2016 Azadmehr_2021). Variants located in the overlapping deleted region (ex: p.Phe312Ser, p.Ser554Gly, p.Arg602Gly) have been classified pathogenic internally. These data indicate that the variant is very likely associated with disease. The following publications have been ascertained in the context of this evaluation (PMID: 35014236, 26897466, 24975702). No submitters have cited clinical-significance assessments for this variant to ClinVar after 2014. Based on the evidence outlined above, the variant was classified as pathogenic. |