Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV002511529 | SCV002820921 | uncertain significance | Glanzmann thrombasthenia | 2022-09-20 | reviewed by expert panel | curation | The NM_000419.5 (ITGA2B):c.1211-78A>G intronic variant occurs at a nucleotide that is modestly conserved (phyloP score 0.988) but is not predicted by SpliceAI (or varSEAK) to have an impact splicing (BP7). It has been reported heterozygous in potential GT patient MB (PMID: 11798398; insufficient to meet any criteria). This variant is absent from gnomAD v2.1.1 (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: BP7, PM2_supporting. |