Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV002510970 | SCV002820929 | uncertain significance | Glanzmann thrombasthenia | 2022-08-05 | reviewed by expert panel | curation | The NM_000212.3(ITGB3):c.856G>C (p.Gly286Arg) missense variant has been reported in at least one homozygous patient with abnormal bleeding (PMID: 31064749). This variant is absent from gnomAD v2.1.1 (PM2_Supporting). The computational predictor REVEL gives a score of 0.969 (PP3). The same amino acid change, resulting from a different nucleotide change c.856G>A, has been reported in a patient with Glanzmann thrombasthenia (PMIDs: 32237906, 21113249) and classified as Likely Pathogenic (PS1_moderate). In summary, this variant meets the criteria to be classified as uncertain significance for autosomal recessive Glanzmann Thrombasthenia based on the ACMG/AMP criteria applied, as specified by the ClinGen PD VCEP: PS1_moderate, PM2_supporitng, PP3. (VCEP specifications version 2; date of approval 08/04/2022). |
NIHR Bioresource Rare Diseases, |
RCV000851903 | SCV000899968 | likely pathogenic | Abnormal bleeding | 2019-02-01 | criteria provided, single submitter | research |