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Impact of treatment for adolescent and young adults with essential thrombocythemia and polycythemia vera
Y. Beauverd, JC. Ianotto, KH. Thaw, M. Sobas, P. Sadjadian, N. Curto-Garcia, LY. Shih, T. Devos, D. Krochmalczyk, S. Galli, M. Bieniaszewska, I. Seferynska, MF. McMullin, A. Armatys, A. Spalek, J. Waclaw, MT. Zdrenghea, L. Legros, F. Girodon, K....
Jazyk angličtina Země Anglie, Velká Británie
Typ dokumentu časopisecké články
- MeSH
- dospělí MeSH
- esenciální trombocytemie * farmakoterapie komplikace terapie MeSH
- kalretikulin genetika MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mutace MeSH
- následné studie MeSH
- polycythaemia vera * farmakoterapie komplikace terapie MeSH
- primární myelofibróza etiologie epidemiologie MeSH
- prognóza MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- trombóza etiologie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Essential 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 × 109/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.
Biology of Cardiovascular Diseases University of Bordeaux INSERM UMR1034 Pessac France
Cellular Biology Department INSERM UMRS 1131 St Louis Hospital APHP Paris France
Centre Hospitalier Universitaire de Brest Brest France
Department of Hematology Amsterdam University Medical Centers Amsterdam Netherlands
Department of Hematology Annecy Genevois Hospital Pringy France
Department of Hematology Collegium Medicum in Bydgoszcz Nicolaus Copernicus University Torun Poland
Department of Hematology Collegium Medicum Jagiellonian University Krakow Poland
Department of Hematology Holy Cross Oncology Center Kielce Poland
Department of Hematology Medical University Lodz Poland
Department of Hematology St Vincent De Paul Hospital Lille France
Department of Hematology State Hospital Opole Poland
Department of Medical Oncology and Hematology University Hospital Zurich Zurich Switzerland
Guy's and St Thomas' NHS Foundation Trust London ENG United Kingdom
Haematology Belfast City Hospital Queen's University Belfast Belfast United Kingdom
Haematology Department Guy's and St Thomas' NHS Foundation Trust London United Kingdom
Hematology and Bone Marrow Transplantation Department University of Medical Sciences Poznan Poland
Hematology and Transplantation Department Medical University and Clinical Center Gdansk Poland
Hematology Department AP HP University of Paris Saclay Bicêtre Hospital Paris France
Hematology Department Hospital del Mar Hospital del Mar Research Institute Barcelona Spain
Hematology Department Jagiellonian University Hospital Krakow Poland
Hematology Department Le Mans Hospital Le Mans France
Hematology Department University Hospital CHUAC A Coruña Spain
Hematology Department University Hospital Linkoping Sweden
Hopital Saint Louis Paris France
Institute of Hematology and Blood Transfusion Prague Prague Czech Republic
Institute of Hematology and Transfusion Medicine Warsaw Poland
Iuliu Hatieganu University of Medicine and Pharmacy Department of Hematology Cluj Napoca Romania
Laboratory of Biological Hematology University Hospital Dijon France
Servicio de Hematología Hospital Universitario Lucus Augusti Lugo Spain
Citace poskytuje Crossref.org
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- $a Beauverd, Yan $u Hematology Division and Faculty of Medicine, Geneva University Hospitals, University of Geneva, Geneva, Switzerland $1 https://orcid.org/0000000279714326
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- $a Impact of treatment for adolescent and young adults with essential thrombocythemia and polycythemia vera / $c Y. Beauverd, JC. Ianotto, KH. Thaw, M. Sobas, P. Sadjadian, N. Curto-Garcia, LY. Shih, T. Devos, D. Krochmalczyk, S. Galli, M. Bieniaszewska, I. Seferynska, MF. McMullin, A. Armatys, A. Spalek, J. Waclaw, MT. Zdrenghea, L. Legros, F. Girodon, K. Lewandowski, B. Bellosillo, J. Samuelsson, A. Abuin Blanco, P. Cony-Makhoul, A. Collins, C. James, R. Kusec, M. Lauermannova, MS. Noya, M. Skowronek, L. Szukalski, A. Szmigielska-Kaplon, M. Wondergem, I. Dudchenko, J. Gora-Tybor, K. Laribi, A. Kulikowska de Nałęcz, JL. Demory, K. Le Dû, S. Zweegman, C. Besses Raebel, RC. Skoda, S. Giraudier, M. Griesshammer, JJ. Kiladjian, CN. Harrison
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