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Despite recent progress, the mechanisms underlying platelet dysfunction in ETV6\u2010RT remain unclear. We investigated 12 patients from six families using functional assays, electron microscopy, quantitative proteomics and cytoskeletal imaging. Most patients exhibited mild\u2010to\u2010moderate thrombocytopenia with variable paediatric bleeding symptoms (median International Society on Thrombosis and Haemostasis Bleeding Assessment Tool 3, range 1\u20139) but consistently mild bleeding in adulthood (median 0, range 0\u20131). Ex\u00a0vivo thrombus formation was reduced independent of platelet count. Electron microscopy revealed defective platelet shape maintenance, characterized by spheroid morphology, reduced\n                    <jats:italic>D<\/jats:italic>\n                    <jats:sub>max<\/jats:sub>\n                    \/\n                    <jats:italic>D<\/jats:italic>\n                    <jats:sub>min<\/jats:sub>\n                    ratios and diminished alpha\u2010granule pools. Flow cytometry and single\u2010platelet total internal reflection fluorescence imaging demonstrated largely preserved calcium signalling but impaired activation\u2010dependent shape change, dense\u2010granule release and integrin activation. Proteomics showed reduced alpha\u2010granule and lysosomal proteins alongside imbalanced regulators of actin remodelling and \u03b2\u2010tubulin. Phalloidin staining confirmed impaired actin cytoskeletal remodelling with reduced lamellipodia formation, while immunofluorescence revealed abnormal \u03b21\u2010tubulin localization with disrupted marginal bands and reduced lysosome\u2010associated membrane protein 1 (LAMP1) expression. Additionally, granulocyte recruitment and migration within thrombi were impaired, suggesting broader thromboinflammatory defects. These findings suggest that combined disruption of cytoskeletal integrity and granule biogenesis underlies impaired thrombus formation in ETV6\u2010RT, providing mechanistic insight into the haemostatic defects associated with this disorder.\n                  <\/jats:p>","DOI":"10.1111\/bjh.70495","type":"journal-article","created":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T00:06:33Z","timestamp":1777421193000},"page":"1965-1979","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Altered cytoskeletal integrity underlies impaired platelet shape change and defective thrombus formation in\n                    <scp>ETV6<\/scp>\n                    \u2010related thrombocytopenia"],"prefix":"10.1111","volume":"208","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9366-3449","authenticated-orcid":false,"given":"Ivan P.","family":"Tesakov","sequence":"first","affiliation":[{"name":"Department of Oncology, 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