Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001225280 | SCV001397549 | uncertain significance | Glanzmann thrombasthenia | 2023-08-15 | reviewed by expert panel | curation | The NM_000419.5(ITGA2B):c.1043G>A (p.Arg348Gln) missense variant is not currently reported in any individuals in the literature, to the best of our knowledge. It is at an extremely low frequency (below the <1/10,000 threshold) with an overall allele frequency from gnomADv2.1.1 of 0.000009297 and a MAF of 0.00007458 (2/26,818 alleles) in the South Asian population (PM2_supporting). REVEL predicts no significant impact of this variant with a score 0.118 (below the <0.25 threshold; BP4). In summary, this variant meets the criteria to be classified as 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, BP4 (PD VCEP specifications version 2.1). |
Division of Human Genetics, |
RCV000477932 | SCV000536919 | uncertain significance | Platelet-type bleeding disorder 16; Glanzmann thrombasthenia | 2016-08-06 | no assertion criteria provided | research |