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Impact of treatment for adolescent and young adults with essential thrombocythemia and polycythemia vera.

dc.rights.licenseopenen_US
dc.contributor.authorBEAUVERD, Yan
dc.contributor.authorIANOTTO, Jean-Christophe
dc.contributor.authorTHAW, Kyaw Htin
dc.contributor.authorSOBAS, Marta
dc.contributor.authorSADJADIAN, Parvis
dc.contributor.authorCURTO-GARCIA, Natalia
dc.contributor.authorSHIH, Lee-Yung
dc.contributor.authorDEVOS, Timothy
dc.contributor.authorKROCHMALCZYK, Dorota
dc.contributor.authorGALLI, Serena
dc.contributor.authorBIENIASZEWSKA, Maria
dc.contributor.authorSEFERYNSKA, Ilona
dc.contributor.authorMCMULLIN, Mary Frances
dc.contributor.authorARMATYS, Anna
dc.contributor.authorSPALEK, Adrianna
dc.contributor.authorWACLAW, Joanna
dc.contributor.authorZDRENGHEA, Mihnea Tudor
dc.contributor.authorLEGROS, Laurence
dc.contributor.authorGIRODON, Francois
dc.contributor.authorLEWANDOWSKI, Krzysztof
dc.contributor.authorBELLOSILLO, Beatriz
dc.contributor.authorSAMUELSSON, Jan
dc.contributor.authorABUIN BLANCO, Aitor
dc.contributor.authorCONY-MAKHOUL, Pascale
dc.contributor.authorCOLLINS, Angela
hal.structure.identifierBiologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases
dc.contributor.authorJAMES, Chloe
dc.contributor.authorKUSEC, Rajko
dc.contributor.authorLAUERMANNOVA, Marie
dc.contributor.authorNOYA, Maria Soledad
dc.contributor.authorSKOWRONEK, Malgorzata
dc.contributor.authorSZUKALSKI, Lukasz
dc.contributor.authorSZMIGIELSKA-KAPLON, Anna
dc.contributor.authorWONDERGEM, Marielle
dc.contributor.authorDUDCHENKO, Iryna
dc.contributor.authorGORA-TYBOR, Joanna
dc.contributor.authorLARIBI, Kamel
dc.contributor.authorKULIKOWSKA DE NAŁĘCZ, Anna
dc.contributor.authorDEMORY, Jean-Loup
dc.contributor.authorLE DÛ, Katell
dc.contributor.authorZWEEGMAN, Sonja
dc.contributor.authorBESSES RAEBEL, Carlos
dc.contributor.authorSKODA, Radek C
dc.contributor.authorGIRAUDIER, Stephane
dc.contributor.authorGRIESSHAMMER, Martin
dc.contributor.authorKILADJIAN, Jean-Jacques
dc.contributor.authorHARRISON, Claire N
dc.date.accessioned2025-05-05T09:59:06Z
dc.date.available2025-05-05T09:59:06Z
dc.date.issued2025-03-12
dc.identifier.issn1476-5551en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/206543
dc.description.abstractEnEssential thrombocythemia (ET) and polycythemia vera (PV) are rare in adolescent and young adult (AYA). These conditions, similar to those in older patients, are linked with thrombotic complications and the potential progression to secondary myelofibrosis (sMF). This retrospective study of ET and PV patients diagnosed before age 25 evaluated complication rates and impact of cytoreductive drugs on outcomes. Among 348 patients (278 ET, 70 PV) with a median age of 20 years, the of thrombotic events was 1.9 per 100 patient-years. Risk factors for thrombosis included elevated white blood cell count (>11 × 10/L) (HR: 2.7, p = 0.012) and absence of splenomegaly at diagnosis (HR: 5.7, p = 0.026), while cytoreductive drugs did not reduce this risk. The incidence of sMF was 0.7 per 100 patient-years. CALR mutation (HR: 6.0, p < 0.001) and a history of thrombosis (HR: 3.8, p = 0.015) were associated with sMF risk. Interferon as a first-line treatment significantly improved myelofibrosis-free survival compared to other treatments or the absence of cytoreduction (p = 0.046). Although cytoreduction did not affect thrombotic event, early interferon use reduced sMF risk. These findings support interferon use to mitigate sMF risk in AYA ET and PV patients.
dc.language.isoENen_US
dc.rightsAttribution 3.0 United States*
dc.rights.urihttp://creativecommons.org/licenses/by/3.0/us/*
dc.title.enImpact of treatment for adolescent and young adults with essential thrombocythemia and polycythemia vera.
dc.title.alternativeLeukemiaen_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1038/s41375-025-02545-2en_US
dc.subject.halSciences du Vivant [q-bio]/Médecine humaine et pathologieen_US
dc.identifier.pubmed40074852en_US
bordeaux.journalLeukemiaen_US
bordeaux.hal.laboratoriesBiologie des maladies cardiovasculaires (BMC) - UMR 1034en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.institutionINSERMen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
bordeaux.import.sourcepubmed
hal.identifierhal-05055971
hal.version1
hal.date.transferred2025-05-05T09:59:14Z
hal.popularnonen_US
hal.audienceInternationaleen_US
hal.exporttrue
workflow.import.sourcepubmed
dc.rights.ccPas de Licence CCen_US
bordeaux.COinSctx_ver=Z39.88-2004&amp;rft_val_fmt=info:ofi/fmt:kev:mtx:journal&amp;rft.jtitle=Leukemia&amp;rft.date=2025-03-12&amp;rft.eissn=1476-5551&amp;rft.issn=1476-5551&amp;rft.au=BEAUVERD,%20Yan&amp;IANOTTO,%20Jean-Christophe&amp;THAW,%20Kyaw%20Htin&amp;SOBAS,%20Marta&amp;SADJADIAN,%20Parvis&amp;rft.genre=article


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