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Of 630 MPN patients, 171 (27%) were PAYA with an average age at diagnosis of 31\u2009years. Females were more prevalent in PAYA than OA (71% vs 58%; <jats:italic>p<\/jats:italic>\u00a0=\u00a00.002), and PAYA more frequently presented with essential thrombocytosis (ET) at diagnosis (67% vs 39%; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). The presence of a <jats:italic>JAK2<\/jats:italic> somatic mutation was higher in OA (80.4% vs 64.3%; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), while a <jats:italic>CALR<\/jats:italic> mutation or lack of any traditional driver mutation was more common in PAYA (20.5% vs 10.5%; <jats:italic>p<\/jats:italic>\u00a0=\u00a00.001, 8.8% vs 3.7%; <jats:italic>p<\/jats:italic>\u00a0=\u00a00.01 respectively). Venous thrombosis was more common in PAYA compared to OA (19.8% vs 10.7%; <jats:italic>p<\/jats:italic>\u00a0=\u00a00.002). PAYA had a higher prevalence of familial MPN and familial cancer predisposition, and two PAYA patients harboured pathogenic germline <jats:italic>JAK2<\/jats:italic> lesions. PAYA demonstrated longer survival from diagnosis than OA (median not reached vs 13\u2009years), while disease transformation was less frequent (19.3% vs 37.9%).<\/jats:p>","DOI":"10.1111\/bjh.18650","type":"journal-article","created":{"date-parts":[[2023,1,17]],"date-time":"2023-01-17T06:06:53Z","timestamp":1673935613000},"page":"449-458","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Characterization of myeloproliferative neoplasms in the paediatric and young adult population"],"prefix":"10.1111","volume":"201","author":[{"given":"Zoey","family":"Harris","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Medicine Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah","family":"Kaizer","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine Johns Hopkins 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