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Real-world study of...
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Sobas, MartaWroclaw Med Univ, Poland
(author)
Real-world study of children and young adults with myeloproliferative neoplasms: identifying risks and unmet needs
- Article/chapterEnglish2022
Publisher, publication year, extent ...
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2022-09-06
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ELSEVIER,2022
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printrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:liu-190239
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https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-190239URI
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https://doi.org/10.1182/bloodadvances.2022007201DOI
Supplementary language notes
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Language:English
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Summary in:English
Part of subdatabase
Classification
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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Funding Agencies|Novartis; Abbvie; Pharmaessentia; Takeda; Jansen; Iqone; Astellas; Astra; Beigene; Celgene; Constellation
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Myeloproliferative 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 = .016) and hyperviscosity symptoms (visual disturbances, dizziness, vertigo, headache) as risk factors (P = .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) (P= .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.
Subject headings and genre
Added entries (persons, corporate bodies, meetings, titles ...)
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Kiladjian, Jean-JacquesUniv Paris, France
(author)
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Beauverd, YanGeneva Univ Hosp, Switzerland
(author)
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Curto-Garcia, NataliaGuys & St Thomas Natl Hlth Serv Fdn Trust, England
(author)
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Sadjadian, ParvisUniv Bochum, Germany
(author)
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Shih, Lee YungChang Gung Mem Hosp, Taiwan
(author)
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Devos, TimothyKatholieke Univ Leuven, Belgium; Katholieke Univ Leuven, Belgium
(author)
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Krochmalczyk, DorotaJagiellonian Univ, Poland
(author)
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Galli, SerenaUniv Hosp, Switzerland; Univ Basel, Switzerland
(author)
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Bieniaszewska, MariaMed Univ & Clin Ctr, Poland
(author)
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Seferynska, IlonaInst Hematol & Transfus Med, Poland
(author)
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McMullin, Mary FrancesQueens Univ Belfast, North Ireland
(author)
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Armatys, AnnaJagiellonian Univ Hosp, Poland
(author)
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Spalek, AdriannaJagiellonian Univ Hosp, Poland
(author)
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Waclaw, JoannaJagiellonian Univ, Poland
(author)
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Zdrenghea, MihneaIuliu Hatieganu Univ Med & Pharm, Romania
(author)
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Legros, LaurenceHop Paul Brousse, France
(author)
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Girodon, FrancoisUniv Hosp, France
(author)
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Lewandowski, KrzysztofUniv Med Sci, Poland
(author)
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Figueras, Anna AngonaHosp del Mar, Spain
(author)
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Samuelsson, JanRegion Östergötland, Hematologiska kliniken US(Swepub:liu)n/a
(author)
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Blanco, Aitor AbuinUniv Hosp, Spain
(author)
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Cony-Makhoul, PascaleAnnecy Genevois Hosp Ctr, France
(author)
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Collins, AngelaNorfolk & Norwich Univ Hosp Natl Hlth Serv Trust, England
(author)
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James, ChloeUniv Bordeaux, France
(author)
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Kusec, RajkoUniv Zagreb, Croatia
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Lauermannova, MarieInst Hematol & Blood Transfus, Czech Republic
(author)
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Noya, Maria SolComplejo Hosp Univ A Coruna, Spain
(author)
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Skowronek, MalgorzataHoly Cross Oncol Ctr, Poland
(author)
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Szukalski, LukaszUniv Torun, Poland
(author)
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Szmigielska-Kaplon, AnnaMed Univ, Poland
(author)
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Wondergem, MarielleUniv Amsterdam, Netherlands
(author)
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Dudchenko, IrynaSumy State Univ, Ukraine
(author)
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Tybor, Joanna GoraMed Univ, Poland
(author)
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Laribi, KamelLe Mans Hosp, France
(author)
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de Nalecz, Anna KulikowskaState Hosp, Poland
(author)
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Demory, Jean-LoupSt Vincent De Paul Hosp, France
(author)
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Le Du, KatellThe Confluent Private Hosp, France
(author)
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Zweegman, SonjaUniv Med Ctr, Netherlands
(author)
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Raebel, Carlos BessesHosp del Mar, Spain
(author)
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Skoda, RadekUniv Hosp, Switzerland; Univ Basel, Switzerland
(author)
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Giraudier, StephaneSt Louis Hosp, France
(author)
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Griesshammer, MartinUniv Bochum, Germany
(author)
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Harrison, Claire N.Guys & St Thomas Natl Hlth Serv Fdn Trust, England
(author)
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Ianotto, Jean-ChristopheUniv Hosp, France
(author)
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Wroclaw Med Univ, PolandUniv Paris, France
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In:BLOOD ADVANCES: ELSEVIER6:17, s. 5171-51832473-95292473-9537
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Sobas, Marta
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Kiladjian, Jean- ...
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Beauverd, Yan
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Sadjadian, Parvi ...
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Shih, Lee Yung
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Devos, Timothy
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Krochmalczyk, Do ...
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McMullin, Mary F ...
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Armatys, Anna
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Spalek, Adrianna
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Waclaw, Joanna
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Zdrenghea, Mihne ...
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Legros, Laurence
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Girodon, Francoi ...
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Lewandowski, Krz ...
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Figueras, Anna A ...
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Samuelsson, Jan
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Blanco, Aitor Ab ...
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Cony-Makhoul, Pa ...
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Collins, Angela
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James, Chloe
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Kusec, Rajko
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Lauermannova, Ma ...
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Noya, Maria Sol
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Skowronek, Malgo ...
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Szukalski, Lukas ...
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Szmigielska-Kapl ...
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Dudchenko, Iryna
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Tybor, Joanna Go ...
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Laribi, Kamel
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de Nalecz, Anna ...
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Demory, Jean-Lou ...
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Le Du, Katell
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Zweegman, Sonja
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Raebel, Carlos B ...
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Giraudier, Steph ...
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and Hematology
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Linköping University