Age-related differences in disease characteristics and clinical outcomes in polycythemia vera

References

• Baxter EJ, Scott LM, Campbell PJ, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet 2005;365:1054–1061.
   PubMed Web of Science ®Google Scholar
• James C, Ugo V, Le Couedic JP, et al. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature 2005;434:1144–1148.
   PubMed Web of Science ®Google Scholar
• Kralovics R, Passamonti F, Buser AS, et al. A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med 200528;352:1779–1790.
   PubMed Web of Science ®Google Scholar
• Levine RL, Wadleigh M, Cools J, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell 2005;7: 387–397.
   PubMed Web of Science ®Google Scholar
• Vardiman JW, Thiele J, Arber DA, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 2009;114: 937–951.
   PubMed Web of Science ®Google Scholar
• Rollison DE, Howlader N, Smith MT, et al. Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001-2004, using data from the NAACCR and SEER programs. Blood 2008;112:45–52.
   PubMed Web of Science ®Google Scholar
• Passamonti F, Rumi E, Pungolino E, et al. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. Am J Med 2004;117:755–761.
   PubMed Web of Science ®Google Scholar
• Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol 2005;23:2224–2232.
   PubMed Web of Science ®Google Scholar
• Landolfi R, Marchioli R, Kutti J, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med 2004;350:114–124.
   PubMed Web of Science ®Google Scholar
• Alvarez-Larran A, Bellosillo B, Martinez-Aviles L, et al. Postpolycythaemic myelofibrosis: frequency and risk factors for this complication in 116 patients. Br J Haematol 2009;146:504–509.
   PubMed Web of Science ®Google Scholar
• Finazzi G, Caruso V, Marchioli R, et al. Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study. Blood 2005;105:2664–2670.
   PubMed Web of Science ®Google Scholar
• Osgood EE. Polycythemia vera: age relationships and survival. Blood 1965;26:243–256.
   PubMed Web of Science ®Google Scholar
• Teofili L, Giona F, Martini M, et al. Markers of myeloproliferative diseases in childhood polycythemia vera and essential thrombocythemia. J Clin Oncol 2007;25:1048–1053.
   PubMed Web of Science ®Google Scholar
• Teofili L, Foa R, Giona F, et al. Childhood polycythemia vera and essential thrombocythemia: does their pathogenesis overlap with that of adult patients?Haematologica 2008;93:169–172.
   PubMed Web of Science ®Google Scholar
• Cario H, McMullin MF, Pahl HL. Clinical and hematological presentation of children and adolescents with polycythemia vera. Ann Hematol 2009;88:713–719.
   PubMed Web of Science ®Google Scholar
• Frezzato M, Ruggeri M, Castaman G, et al. Polycythemia vera and essential thrombocythemia in young patients. Haematologica 1993;78:11–17.
   PubMedGoogle Scholar
• Najean Y, Mugnier P, Dresch C, et al. Polycythaemia vera in young people: an analysis of 58 cases diagnosed before 40 years. Br J Haematol 1987;67:285–291.
   PubMed Web of Science ®Google Scholar
• Passamonti F, Malabarba L, Orlandi E, et al. Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia. Haematologica 2003;88:13–18.
   PubMed Web of Science ®Google Scholar
• Perea G, Remacha A, Besses C, et al. Is polycythemia vera a serious disease in young adults?Haematologica 2001;86:543–544.
   PubMed Web of Science ®Google Scholar
• Ruggeri M, Finotto S, Fortuna S, et al. Treatment outcome in a cohort of young patients with polycythemia vera. Intern Emerg Med 2010;5:411–413.
   PubMed Web of Science ®Google Scholar
• Berk PD, Goldberg JD, Donovan PB, et al. Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Semin Hematol 1986;23:132–143.
   PubMed Web of Science ®Google Scholar
• Mesa RA, Verstovsek S, Cervantes F, et al. Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): consensus on terminology by the international working group for myelofibrosis research and. reatment (IWG-MRT). Leuk Res 2007;31:737–740.
   PubMed Web of Science ®Google Scholar
• Moliterno AR, Williams DM, Rogers O, et al. Molecular mimicry in the chronic myeloproliferative disorders: reciprocity between quantitative JAK2 V617F and Mpl expression. Blood 2006;108:3913–3915.
   PubMed Web of Science ®Google Scholar
• Videbaek A. Polycythaemia vera. Course and prognosis. Acta Med Scand 1950;138:179–187.
   PubMed Web of Science ®Google Scholar
• Giona F, Teofili L, Moleti ML, et al. Thrombocythemia and polycythemia in patients younger than 20 years at diagnosis: clinical and biologic features, treatment, and long-term outcome. Blood 2012;119:2219–2227.
   PubMed Web of Science ®Google Scholar
• Landgren O, Goldin LR, Kristinsson SY, et al. Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24,577 first-degree relatives of 11,039 patients with myeloproliferative neoplasms in Sweden. Blood 2008;112:2199–2204.
   PubMed Web of Science ®Google Scholar
• Rumi E, Passamonti F, Della Porta MG, et al. Familial chronic myeloproliferative disorders: clinical phenotype and evidence of disease anticipation. J Clin Oncol 2007;25:5630–5635.
   PubMed Web of Science ®Google Scholar
• Stein BL, Williams DM, Wang NY, et al. Sex differences in the JAK2 V617F allele burden in chronic myeloproliferative disorders. Haematologica 2010;95:1090–1097.
   PubMed Web of Science ®Google Scholar
• Stein BL, Rademaker A, Spivak JL, et al. Gender and vascular complications in the JAK2 V617F-positive myeloproliferative neoplasms. Thrombosis 2011;2011:874146.
   PubMedGoogle Scholar
• Colaizzo D, Amitrano L, Tiscia GL, et al. The JAK2 V617F mutation frequently occurs in patients with portal and mesenteric venous thrombosis. J Thromb Haemost 2007;5:55–61.
   PubMed Web of Science ®Google Scholar
• Colaizzo D, Tiscia GL, Bafunno V, et al. Sex modulation of the occurrence of jak2 v617f mutation in patients with splanchnic venous thrombosis. Thromb Res 2011;128:233–236.
   PubMed Web of Science ®Google Scholar
• Kiladjian JJ, Cervantes F, Leebeek FW, et al. The impact of JAK2 and MPL mutations on diagnosis and prognosis of splanchnic vein thrombosis: a report on 241 cases. Blood 2008;111:4922–4929.
   PubMed Web of Science ®Google Scholar
• Landolfi R, Di Gennaro L, Nicolazzi MA, et al. Polycythemia vera: gender-related phenotypic differences. Intern Emerg Med 2012;7:509–515.
   PubMed Web of Science ®Google Scholar
• Passamonti F, Rumi E, Pietra D, et al. A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications. Leukemia 2010;24:1574–1579.
   PubMed Web of Science ®Google Scholar
• Gangat N, Strand J, Li CY, et al. Leucocytosis in polycythaemia vera predicts both inferior survival and leukaemic transformation. Br J Haematol 2007;138:354–358.
   PubMed Web of Science ®Google Scholar
• Landolfi R, Di Gennaro L, Barbui T, et al. Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood 2007;109:2446–2452.
   PubMed Web of Science ®Google Scholar
• Vannucchi AM, Antonioli E, Guglielmelli P, et al. Prospective identification of high-risk polycythemia vera patients based on JAK2(V617F) allele burden. Leukemia 2007;21:1952–1959.
   PubMed Web of Science ®Google Scholar
• De Stefano V, Za T, Rossi E, et al. Increased risk of recurrent thrombosis in patients with essential thrombocythemia carrying the homozygous JAK2 V617F mutation. Ann Hematol 2010;89:141–146.
   PubMed Web of Science ®Google Scholar
• Primignani M, Barosi G, Bergamaschi G, et al. Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis. Hepatology 2006;44:1528–1534.
   PubMed Web of Science ®Google Scholar
• Villani L, Bergamaschi G, Primignani M, et al. JAK2 46/1 haplotype predisposes to splanchnic vein thrombosis-associated BCR-ABL negative classic myeloproliferative neoplasms. Leuk Res 2012;36: e7–e9.
   Google Scholar
• Smalberg JH, Koehler E, Darwish Murad S, et al. The JAK2 46/1 haplotype in Budd-Chiari syndrome and portal vein thrombosis. Blood 201114;117:3968–3973.
   PubMed Web of Science ®Google Scholar
• Kouroupi E, Kiladjian JJ, Chomienne C, et al. The JAK2 46/1 haplotype in splanchnic vein thrombosis. Blood 2011;117:5777–5778.
   PubMed Web of Science ®Google Scholar
• Passamonti F, Rumi E, Caramella M, et al. A dynamic prognostic model to predict survival in post-polycythemia vera myelofibrosis. Blood 2008;111:3383–3387.
   PubMed Web of Science ®Google Scholar
• Silver RT, Vandris K, Wang YL, et al. JAK2(V617F) allele burden in polycythemia vera correlates with grade of myelofibrosis, but is not substantially affected by therapy. Leuk Res 2011;35:177–182.
   PubMed Web of Science ®Google Scholar
• Kiladjian JJ, Cassinat B, Chevret S, et al. Pegylated interferon- alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera. Blood 2008;112:3065–3072.
   PubMed Web of Science ®Google Scholar
• Quintas-Cardama A, Kantarjian H, Manshouri T, et al. Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera. J Clin Oncol 2009;27:5418–5424.
   PubMed Web of Science ®Google Scholar
• Verstovsek S, Passamonti F, Rambaldi A, et al. Durable responses with the JAK1/JAK2 inhibitor, INCB018424, in patients with polycythemia vera (PV) and essential thrombocythemia (ET) refractory or intolerant to hydroxyurea (HU). Blood 2010;116: Abstract 313.
   Web of Science ®Google Scholar
• Hasselbalch HC. A new era for IFN-alpha in the treatment of Philadelphia-negative chronic myeloproliferative neoplasms. Expert Rev Hematol 2011;4:637–655.
   PubMed Web of Science ®Google Scholar