Age-related differences in disease characteristics and clinical outcomes in polycythemia vera
- 8 January 2013
- journal article
- Published by Informa UK Limited in Leukemia & Lymphoma
- Vol. 54 (9), 1989-1995
- https://doi.org/10.3109/10428194.2012.759656
Abstract
The natural history and prognosis for young patients with polycythemia vera (PV) in the post-JAK2 V617F era are not well defined. Therefore, we retrospectively analyzed disease characteristics and clinical outcomes in 120 patients ≤ 45 years and 84 patients ≥ 65 years at diagnosis. Despite lower white blood counts (9.2 vs. 13.4 × 109/L, p = 0.004) and a lower JAK2 V617F allele burden (51% vs. 66%, p = 0.015), younger patients with PV had comparable rates of vascular complications compared to older patients (27% vs. 31%, p = 0.64). However, splanchnic vein thrombosis occurred more frequently in younger patients (13% vs. 2%, p = 0.0056). Myelofibrotic and leukemic transformation, the most serious complications of myeloproliferative neoplasms (MPN), occurred with similar frequencies in young versus older patients (15% vs. 10%, p = 0.29). Prevention or delay of these complications is currently the most urgent challenge in the care of younger patients with PV.Keywords
This publication has 43 references indexed in Scilit:
- Postpolycythaemic myelofibrosis: frequency and risk factors for this complication in 116 patientsBritish Journal of Haematology, 2009
- The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changesBlood, 2009
- Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001-2004, using data from the NAACCR and SEER programsBlood, 2008
- A Gain-of-Function Mutation ofJAK2in Myeloproliferative DisordersNew England Journal of Medicine, 2005
- Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosisCancer Cell, 2005
- Vascular and Neoplastic Risk in a Large Cohort of Patients With Polycythemia VeraJournal of Clinical Oncology, 2005
- A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia veraNature, 2005
- Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disordersThe Lancet, 2005
- Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemiaAmerican Journal Of Medicine, 2004
- Efficacy and Safety of Low-Dose Aspirin in Polycythemia VeraNew England Journal of Medicine, 2004