Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV002264772 | SCV002546413 | pathogenic | Hereditary thrombocytopenia and hematologic cancer predisposition syndrome | 2022-01-22 | reviewed by expert panel | curation | The NM_001754.5(RUNX1):c.317G>A (p.Trp106Ter) variant is a nonsense variant that is predicted to introduce a premature stop codon and expected to result in nonsense-mediated mRNA decay (PVS1). This variant is completely absent from all population databases with at least 20x coverage for RUNX1 (PM2_Supporting). The variant has been reported in one proband meeting at least one of the RUNX1-phenotypic criteria (PS4_Supporting; PMID: 24100448). In summary, this variant meets criteria to be classified as pathogenic. ACMG/AMP criteria applied, as specified by the Myeloid Malignancy Variant Curation Expert Panel for RUNX1: PVS1, PM2_Supporting and PS4_Supporting. |
Birmingham Platelet Group; University of Birmingham | RCV001270532 | SCV001450831 | pathogenic | Abnormal bleeding; Thrombocytopenia | 2020-05-01 | no assertion criteria provided | research |