Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001290501 | SCV001478541 | uncertain significance | Glanzmann thrombasthenia | 2023-11-02 | reviewed by expert panel | curation | The ITGA2B missense variant NM_000419.5:c.435C>A replaces the asparagine residue with a lysine residue (p.Asn145Lys). This variant has been observed in a proband (PMID 26096001) with a phenotype specific for Glanzmann's thrombasthenia (PP4_Moderate) who also harbors a second ITGA2B variant (c.531T>A, p.Cys177Ter) previously classified by the VCEP as pathogenic (phase unconfirmed; PM3_supporting). This variant is extremely rare in population databases, having been observed in 1/34534 alleles in the Latino population in gnomAD v2.1.1 (PM2_supporting). In summary, this variant is of uncertain significance and lacks sufficient evidence to be classified as pathogenic or benign for Glanzmann thrombasthenia. GT-specific criteria applied: PM2_supporting, PM3_supporting, PP4_moderate. |