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dc.contributor.authorSobas, M.
dc.contributor.authorKiladjian, J.-J.
dc.contributor.authorBeauverd, Y.
dc.contributor.authorCurto-Garcia, N.
dc.contributor.authorSadjadian, P.
dc.contributor.authorShih, L.Y.
dc.contributor.authorDevos, T.
dc.contributor.authorKrochmalczyk, D.
dc.contributor.authorGalli, S.
dc.contributor.authorBieniaszewska, M.
dc.contributor.authorSeferynska, I.
dc.contributor.authorMcMullin, M.F.
dc.contributor.authorArmatys, A.
dc.contributor.authorSpalek, A.
dc.contributor.authorWaclaw, J.
dc.contributor.authorZdrenghea, M.
dc.contributor.authorLegros, L.
dc.contributor.authorGirodon, F.
dc.contributor.authorLewandowski, K.
dc.contributor.authorFigueras, A.A.
dc.contributor.authorSamuelsson, J.
dc.contributor.authorBlanco, A.A.
dc.contributor.authorCony-Makhoul, P.
dc.contributor.authorCollins, A.
dc.contributor.authorJames, C.
dc.contributor.authorKusec, R.
dc.contributor.authorLauermannova, M.
dc.contributor.authorNoya Pereira, María Soledad 
dc.contributor.authorSkowronek, M.
dc.contributor.authorSzukalski, L.
dc.contributor.authorSzmigielska-Kaplon, A.
dc.contributor.authorWondergem, M.
dc.contributor.authorDudchenko, I.
dc.contributor.authorTybor, J.G.
dc.contributor.authorLaribi, K.
dc.contributor.authorde Nalecz, A.K.
dc.contributor.authorDemory, J.-L.
dc.contributor.authorLe Du, K.
dc.contributor.authorZweegman, S.
dc.contributor.authorRaebel, C.B.
dc.contributor.authorSkoda, R.
dc.contributor.authorGiraudier, S.
dc.contributor.authorGriesshammer, M.
dc.contributor.authorHarrison, C.N.
dc.contributor.authorIanotto, J.-C.
dc.date.accessioned2025-08-26T11:08:01Z
dc.date.available2025-08-26T11:08:01Z
dc.date.issued2022
dc.identifier.citationSobas M, Kiladjian J-J, Beauverd Y, Curto-Garcia N, Sadjadian P, Shih LY, et al. Real-world study of children and young adults with myeloproliferative neoplasms: identifying risks and unmet needs. Blood Advances. 2022;6(17):5171-83.
dc.identifier.issn2473-9537
dc.identifier.otherhttps://portalcientifico.sergas.gal/documentos/6522c802ec1a10197ffd9716*
dc.identifier.urihttp://hdl.handle.net/20.500.11940/20857
dc.description.abstractMyeloproliferative neoplasms (MPNs) are uncommon in children/young adults. Here, we present data on unselected patients diagnosed before 25 years of age included from 38 centers in 15 countries. Sequential patients were included. We identified 444 patients, with median follow-up 9.7 years (0-47.8). Forty-nine (11.1%) had a history of thrombosis at diagnosis, 49 new thrombotic events were recorded (1.16% patient per year [pt/y]), perihepatic vein thromboses were most frequent (47.6% venous events), and logistic regression identified JAK2V617F mutation (P 5 .016) and hyperviscosity symptoms (visual disturbances, dizziness, vertigo, headache) as risk factors (P 5 .040). New hemorrhagic events occurred in 44 patients (9.9%, 1.04% pt/y). Disease transformation occurred in 48 patients (10.9%, 1.13% pt/y), usually to myelofibrosis (7.5%) with splenomegaly as a novel risk factor for transformation in essential thrombocythemia (ET) (P5 .000) in logistical regression. Eight deaths (1.8%) were recorded, 3 after allogeneic stem cell transplantation. Concerning conventional risk scores: International Prognostic Score for Essential Thrombocythemia-Thrombosis and new International Prognostic Score for Essential Thrombocythemia-Thrombosis differentiated ET patients in terms of thrombotic risk. Both scores identified high-risk patients with the same median thrombosis-free survival of 28.5 years. No contemporary scores were able to predict survival for young ET or polycythemia vera patients. Our data represents the largest real-world study of MPN patients age, 25 years at diagnosis. Rates of thrombotic events and transformation were higher than expected compared with the previous literature. Our study provides new and reliable information as a basis for prospective studies, trials, and development of harmonized international guidelines for the specific management of young patients with MPN.en
dc.description.sponsorshipM. Sobas reports advisory boardmembership (Novartis, BMS, Clegene) and speaker fees (Novartis,Abbvie). J.-J.K. reports advisory board membership (Novartis, AOPOrphan, BMS, Abbvie, Incyte) and speaker fees (Novartis, Pharmaessentia). M.F.M. reports advisory board membership (Novartis,BMS, Abbvie, Incyte, CTI, Sierra oncology), speaker bureau partici-pation (Novartis, Abbvie, Pfizer, AOP, Incyte), and clinical trial sup-port (BMS, AOP). J.G.T. reports advisory board membership(Novartis, BMS, Celgene, Pfizer) and honoraria (Novartis, BMS,Celgene, Abbvie, Pfizer). K. Laribi reports grants (Novartis, Takeda,Jansen, Abbvie) and personal fees (Novartis, Takeda, Abbvie, Iqone,Astellas, Astra, Beigene). K.L.D. reports honoraria (Abbvie, Incyte,Janssen, Roche,Takeda). M.G. reports consultancy (Amgen, AOPOrphan, Novartis, Celgene, CTI, Shire, Pfizer, Roche, Janssen,Gilead, Astra Zeneca) and honoraria (Amgen, AOP Orphan, Novar-tis, Celgene, CTI, Shire, Pfizer, Roche, Janssen, Gilead, AstraZeneca). C.N.H. reports consultancy and honoraria (AOP Orphan,Novartis, Celgene, CTI, Roche, Janssen, Gilead, Geron, Galecto,Sierra), speaker bureau participation (AOP Orphan, Novartis, Cel-gene, CTI, Geron), and grants (Celgene, Constellation, Novartis). J.-C.I. reports grants, honoraria, and advisory board membership(Novartis). The remaining authors declare no competingfinancialinterests.en
dc.language.isoeng
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International*
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/4.0/
dc.titleReal-world study of children and young adults with myeloproliferative neoplasms: identifying risks and unmet needs*
dc.typeArticleen
dc.authorsophosSobas, J. C. M.
dc.authorsophosKiladjian, J. J.
dc.authorsophosBeauverd, Y.
dc.authorsophosCurto-Garcia, N.
dc.authorsophosSadjadian, P.
dc.authorsophosShih, L. Y.
dc.authorsophosDevos, T.
dc.authorsophosKrochmalczyk, D.
dc.authorsophosGalli, S.
dc.authorsophosBieniaszewska, M.
dc.authorsophosSeferynska, I.
dc.authorsophosMcMullin, M. F.
dc.authorsophosArmatys, A.
dc.authorsophosSpalek, A.
dc.authorsophosWaclaw, J.
dc.authorsophosZdrenghea, M.
dc.authorsophosLegros, L.
dc.authorsophosGirodon, F.
dc.authorsophosLewandowski, K.
dc.authorsophosFigueras, A. A.
dc.authorsophosSamuelsson, J.
dc.authorsophosBlanco, A. A.
dc.authorsophosCony-Makhoul, P.
dc.authorsophosCollins, A.
dc.authorsophosJames, C.
dc.authorsophosKusec, R.
dc.authorsophosLauermannova, M.
dc.authorsophosNoya, M. S.
dc.authorsophosSkowronek, M.
dc.authorsophosSzukalski, L.
dc.authorsophosSzmigielska-Kaplon, A.
dc.authorsophosWondergem, M.
dc.authorsophosDudchenko, I.
dc.authorsophosTybor, J. G.
dc.authorsophosLaribi, K.
dc.authorsophosde Nalecz, A. K.
dc.authorsophosDemory, J. L.
dc.authorsophosLe Du, K.
dc.authorsophosZweegman, S.
dc.authorsophosRaebel, C. B.
dc.authorsophosSkoda, R.
dc.authorsophosGiraudier, S.
dc.authorsophosGriesshammer, M.
dc.authorsophosHarrison, C. N.
dc.authorsophosIanotto
dc.identifier.doi10.1182/bloodadvances.2022007201
dc.identifier.sophos6522c802ec1a10197ffd9716
dc.issue.number17
dc.journal.titleBlood Advances*
dc.page.initial5171
dc.page.final5183
dc.relation.projectIDNovartis; Abbvie; Pharmaessentia; Takeda; Jansen; Iqone; Astellas; Astra; Beigene; Celgene; Constellation
dc.relation.publisherversionhttps://ashpublications.org/bloodadvances/article-pdf/6/17/5171/1918309/advancesadv2022007201.pdf;https://watermark.silverchair.com/advancesadv2022007201.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAABIkwggSFBgkqhkiG9w0BBwagggR2MIIEcgIBADCCBGsGCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQMNmlBXlfgc8x-RzU3AgEQgIIEPMJmEq3aA9g8FZ4780EDbiLMzz-oJ3esXAlVBrMv71BXqm1BLrTlvEgSbgEL9II6loRhEr73HSS9hEe2CL6oHNG5R8eo2EICQFEhXkCrTr66cW-2l0AF2z2hC7jFIFvaWb9tAHGZ-OthxFc0AIIbFz7ii2kzM1l-2njBQCAczP9VeoOGYWcliHfwmJ0XSMgcJkqHMNvbv-QkOv_0B2fiBCUNNOO2-oPONvFC8bgMuEQqzUK0-7g6l-L55drrFPByzqxlhvQnRBFF_-yt05AfPwWUT_BdjlST47TizlQnbZRkUQjsdtGNVu8LrsbnQr-dF86L8RCfF8lNaHEf8Q2uctv50Lwq_fghcVzHG89iGo-ShO7ZY5wjnIoUGlnDxBKh-CdYBIsUKpxGg7zZVWK2BM2xI5_FaaF3tOP4X6iQtQY16_stX3L6N65_tN3Fo0I6hONW1q43HJKwMfkWVXUc7KzonPonoJ1mmFZ48zjBhHKv33zAzPers-y9O0D3IZss6MYLHgG0w4CjsvErwjdocgZxVn0MwHDVIveC2OVUlbxfOjVwfAxp13tYSnd-O22Vg_jNedV-bZOPT_GjHnexdH06dc5d2wvAWFuUMrJoRmUFGYvNdJD3g5_y_LwnRX9lkQgIQjI6OpkKKaP_hrt2iZFoGTSmfPT3ab2M0sSie7rogbCJjXTFlUAtAtNvGZ0ok9F93wFPfJFAwy7NioSb7PcfUbcrrlH43GDKxcvPR2FzkvW2zRFRONERvWKxa161KzCJtwqxJVxfuxTcX0pTIgZmvT9SI4WiVUVl30iVPN18IL1hzeJSjA1TeiVsVj6SNe35nnbV1zi1QbI3GwSdKpZlF4Y5hhsVsRCtsH38M_7PYjw07dqqfshJUm34yIVDdIiGvDIBiSYS-Zbx8R5gPoKE6l6ODdGmG5pVCKlA2uNzxfcYqaBTMU1gVcQHkLR9Brs4Jzuoalqai12x88tt5zXCs_-D2vcMmpgbdbZHKaDsSV6F58EONpjTiJDZNhomnRZmx1LRNt2x9jUJoLhK5dbHSg-vbJ_DmNaWz8oCclnC6cXHnog9Xoq7rD7IBGWoNvAY98O6_RZhaVJoy1mt622Tx42AE80eDpuNejwNrcknyShTWPZmi-645AhbDaflkyj8_poRxNCCMpbF6bV_CPUmh6ChmKL_3bTr7oX3Yzj2v5urxcliAxRezwIKbddFzgu9ouK6nrvjhf5m8MOwFtnzd56HRu2TnlhyxMSd-lmpG574CK9GhuwX-lfL5oWL_m37AkvDCVRt9yHXxRCW2-cfhwBiyTV0AxWb4KICUUfNqH9YsYrW6BNND-qb2iJ3GPM1rBW5ksO_mvQP1GVcUs8k88GhoU0K2Y8AQ8dvlKZQboWAOzLcrmtFb57txhfkUmpQ6VYGMVWYiErTCpnEbiMtZI82EIS3DbDL9MUes
dc.rights.accessRightsopenAccess
dc.subject.keywordAS Coruñaes
dc.subject.keywordCHUACes
dc.subject.keywordINIBICes
dc.typefidesArtículo Científico (incluye Original, Original breve, Revisión Sistemática y Meta-análisis)es
dc.typesophosArtículo Originales
dc.volume.number6


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