Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV002254807 | SCV002525896 | uncertain significance | Glanzmann thrombasthenia | 2022-04-07 | reviewed by expert panel | curation | The NM_000212.3:c.59T>C variant in ITGB3 is a missense variant predicted to cause substitution of leucine by proline at amino acid 20 (p.Leu20Pro). This variant has been observed in homozygosity in one individual (GT10 in PMID: 19691478) (PM3_Supporting). This individual displayed mucocutaneous bleeding and impaired aggregation with all agonists except ristocetin, which is highly specific for Glanzmann thrombasthenia (PP4_Moderate). This variant is absent from gnomAD v2.1.1 (PM2_Supporting). In summary, due to insufficient evidence, this variant is classified as a variant of uncertain significance for autosomal recessive Glanzmann Thrombasthenia based on the ACMG/AMP criteria applied, as specified by the ClinGen PD-VCEP: PM2_Supporting, PM3_Supporting, PP4_Moderate. (VCEP specifications version 2; date of approval 03/15/2022) |