Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001195650 | SCV001366046 | pathogenic | Hereditary thrombocytopenia and hematologic cancer predisposition syndrome | 2024-03-26 | reviewed by expert panel | curation | The NM_001754.4:c.240_241del (p.Glu80fs) variant is a frameshift 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). This variant is a nonsense/frameshift variants that is downstream of c.98 (PM5_Supporting). Two unpublished probands meeting the RUNX1 phenotype criteria are noted (PS4_Moderate; SCV000807768.1). In summary, this variant meets criteria to be classified as likely pathogenic. ACMG/AMP criteria applied, as specified by the Myeloid Malignancy Variant Curation Expert Panel for RUNX1: PVS1, PM2_supporting, PM5_supporting, PS4_Moderate. |
Prevention |
RCV000680397 | SCV000807768 | pathogenic | not provided | 2019-10-06 | criteria provided, single submitter | clinical testing |