Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001225244 | SCV001397496 | likely pathogenic | Glanzmann thrombasthenia | 2023-11-02 | reviewed by expert panel | curation | The c.1458C>G (p.Cys486Trp) variant has been reported in the literature in at least 1 compound heterozygous proband in trans with pathogenic variant Trp11Arg (PMID: 25373348) and a homozygote in an abstract (Von Bargen et al., 2018; PM3). One proband (PMID: 25373348) meets the criteria for PP4_Strong; including mucocutaneous bleeding, impaired aggregation with all agonists except ristocetin, and reduced surface expression of αIIbβ3 measured by flow cytometry. It is absent from gnomADv2.1.1 (PM2_supporting) and is predicted to have a deleterious effect (REVEL score of 0.909; PP3). In summary, this variant meets criteria to be classified as Likely Pathogenic for GT. GT-specific criteria applied: PM2_supporting, PM3, PP3, and PP4_strong. |