Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV003222579 | SCV003916007 | uncertain significance | Glanzmann thrombasthenia | 2023-04-06 | reviewed by expert panel | curation | The NM_000212.3(ITGB3):c.538G>A (p.Gly180Arg) missense variant has been reported in one patient (Patient 5, PMID: 31029159) stated to have clinical diagnosis of Glanzmann thrombasthenia but insufficient information was available to determine if the phenotype is highly specific to GT. It has a REVEL score of 0.958, which is above the ClinGen PD VCEP threshold of >0.7 and predicts a damaging effect on function (PP3). The highest population minor allele frequency in gnomAD v 2.1.1 is 0.00003266 (1/30614 alleles) in the South Asian population, which is lower than the ClinGen PD VCEP threshold (<0.0001; PM2_Supporting). 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: PM2_supporting, PP3 (VCEP specifications version 2.1). |