Age-related differences in disease characteristics and clinical outcomes in polycythemia vera. Issue 9 (September 2013)

• Record Type: Journal Article
• Authors: Stein, Brady L.; Saraf, Santosh; Sobol, Urszula; Halpern, Anna; Shammo, Jamile; Rondelli, Damiano; Michaelis, Laura; Odenike, Olatoyosi; Rademaker, Alfred; Zakarija, Anaadriana; McMahon, Brandon; Spivak, Jerry L.; Moliterno, Alison R.
• Is Part Of: Leukemia & lymphoma. Volume 54:Issue 9(2013:Sep.)
• Page Start: 1989
• Page End: 1995
• Publication Date: 2013-09
• Subjects: Myeloproliferative neoplasm -- polycythemia -- vera -- young -- JAK2 V617F -- splanchnic vein thrombosis; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.3109/10428194.2012.759656
• Languages: English
• ISSNs: 1042-8194

Abstract

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 × 10 9 /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.