Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001285457 | SCV001471885 | likely pathogenic | Hereditary factor VIII deficiency disease | 2020-03-13 | criteria provided, single submitter | clinical testing | The F8 c.5813A>G; p.His1938Arg variant is reported in the literature in multiple individuals affected with mild to moderate hemophilia A (Riccardi 2010, Factor VIII Variant Database and references therein). This variant is absent from general population databases (Exome Variant Server, Genome Aggregation Database), indicating it is not a common polymorphism. The histidine at codon 1938 is highly conserved, computational analyses (SIFT, PolyPhen-2) predict that this variant is deleterious, and functional assays of patient samples with this variant suggest it has between 2% and 30% of wildtype F8 activity (Riccardi 2010, Factor VIII Variant Database). Further, another variant at the same codon (p.His1938Leu) has been reported in individuals with mild hemophilia A and is considered disease-causing (Factor VIII Variant Database). Based on available information, the p.His1938Arg variant is considered to be likely pathogenic. References: Factor VIII Variant Database: http://f8-db.eahad.org/ Riccardi F et al. Spectrum of F8 gene mutations in haemophilia A patients from a region of Italy: identification of 23 new mutations. Haemophilia. 2010 Sep 1;16(5):791-800. |
Mayo Clinic Laboratories, |
RCV003481053 | SCV004225587 | likely pathogenic | not provided | 2022-11-16 | criteria provided, single submitter | clinical testing | PP3, PM1_supporting, PM2, PS4_moderate |