{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T04:23:41Z","timestamp":1783052621709,"version":"3.54.6"},"reference-count":14,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,6,23]],"date-time":"2025-06-23T00:00:00Z","timestamp":1750636800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,6,23]],"date-time":"2025-06-23T00:00:00Z","timestamp":1750636800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000272","name":"National Institute for Health and Care Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    One hundred of 963 consecutive registrants with primary myelofibrosis (PMF) in the Pavia\u2010CSM database had haemoglobin concentration at diagnosis \u2265160\u2009g\/L in females or \u2265\u2009165\u2009g\/L in males. These subjects were more often female and younger; had higher white blood cell (WBC) and platelet concentrations; and higher frequency of\n                    <jats:italic>JAK2<\/jats:italic>\n                    <jats:sup>V617F<\/jats:sup>\n                    and\n                    <jats:italic>JAK2<\/jats:italic>\n                    <jats:sup>V617F<\/jats:sup>\n                    variant allele frequency (VAF) compared with those without increased haemoglobin at diagnosis. They had less active disease defined as smaller spleen, lower plasma lactate dehydrogenase, lower blood CD34\u2010positive cell concentration and less bone marrow fibrosis. They also had a lower plasma high\u2010sensitivity C\u2010reactive protein concentration consistent with less inflammation. These subjects were more likely to evolve towards an increased WBC concentration and had a lower risk of progressing to a myelodepletive phenotype compared with those without increased haemoglobin concentration at diagnosis and longer survival (22 vs. 15\u2009years;\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001). In subjects with increased haemoglobin concentration at diagnosis, age &gt;50\u2009years and\n                    <jats:italic>JAK2<\/jats:italic>\n                    <jats:sup>V617F<\/jats:sup>\n                    VAF\u2009&gt;\u200975% correlated with worse survival. Our findings suggest that PMF with increased haemoglobin concentration at diagnosis is a unique variant characterized by\n                    <jats:italic>JAK2<\/jats:italic>\n                    <jats:sup>V617F<\/jats:sup>\n                    \u2010driven hyperproliferation with less inflammation. These features impact prognosis and therapy.\n                  <\/jats:p>","DOI":"10.1111\/bjh.20233","type":"journal-article","created":{"date-parts":[[2025,6,24]],"date-time":"2025-06-24T02:28:52Z","timestamp":1750732132000},"page":"1930-1937","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Primary myelofibrosis with increased haemoglobin concentration at presentation"],"prefix":"10.1111","volume":"207","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8357-7192","authenticated-orcid":false,"given":"Giovanni","family":"Barosi","sequence":"first","affiliation":[{"name":"Center for the Study of Myelofibrosis, Scientific Direction Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita","family":"Campanelli","sequence":"additional","affiliation":[{"name":"Center for the Study of Myelofibrosis, Scientific Direction Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Catarsi","sequence":"additional","affiliation":[{"name":"Center for the Study of Myelofibrosis, Scientific Direction Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlotta","family":"Abb\u00e0","sequence":"additional","affiliation":[{"name":"General Medicine 2\u2010Center for Systemic Amyloidosis and High\u2010Complexity Diseases Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana","family":"Carolei","sequence":"additional","affiliation":[{"name":"Center for the Study of Myelofibrosis, Scientific Direction Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8037-0439","authenticated-orcid":false,"given":"Margherita","family":"Massa","sequence":"additional","affiliation":[{"name":"General Medicine 2\u2010Center for Systemic Amyloidosis and High\u2010Complexity Diseases Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa","family":"De Silvestri","sequence":"additional","affiliation":[{"name":"Biometry and Clinical Epidemiology, Scientific Direction Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9156-1676","authenticated-orcid":false,"given":"Robert Peter","family":"Gale","sequence":"additional","affiliation":[{"name":"Centre for Haematology Research, Department of Immunology and Inflammation Imperial College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4195-2289","authenticated-orcid":false,"given":"Vittorio","family":"Rosti","sequence":"additional","affiliation":[{"name":"Center for the Study of Myelofibrosis, Scientific Direction Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation  Pavia 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