Treatment options for essential thrombocythemia and polycythemia vera

References

• Tefferi A. The history of myeloproliferative disorders: before and after Dameshek. Leukemia22(1), 3–13 (2008).
   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 treatment (IWG-MRT). Leuk. Res.31(6), 737–740 (2007).
   PubMed Web of Science ®Google Scholar
• Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood100(7), 2292–2302 (2002).
   PubMed Web of Science ®Google Scholar
• Kaushansky K. On the molecular origins of the chronic myeloproliferative disorders: it all makes sense. Blood105(11), 4187–4190 (2005).
   PubMed Web of Science ®Google Scholar
• Tefferi A, Thiele J, Orazi A et al. Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel. Blood110(4), 1092–1097 (2007).
   PubMed Web of Science ®Google Scholar
• Swerdlow SH, Campo E, Harris NL et al. (Eds). WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. International Agency for Research on Cancer, Lyon, France (2008).
   Google Scholar
• Tefferi A, Elliott MA, Pardanani A. Atypical myeloproliferative disorders: diagnosis and management. Mayo Clin. Proc.81(4), 553–563 (2006).
   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: utilizing data from the NAACCR and SEER programs. Blood112(1), 45–52 (2008).
   PubMed Web of Science ®Google Scholar
• Chen GL, Prchal JT. X-linked clonality testing: interpretation and limitations. Blood110(5), 1411–1419 (2007).
   PubMed Web of Science ®Google Scholar
• Skoda R. The genetic basis of myeloproliferative disorders. Hematology Am. Soc. Hematol. Educ. Program1–10 (2007).
   PubMedGoogle Scholar
• Levine RL, Gilliland DG. Myeloproliferative disorders. Blood112(6), 2190–2198 (2008).
   PubMed Web of Science ®Google Scholar
• Vainchenker W, Constantinescu SN. A unique activating mutation in JAK2 (V617F) is at the origin of polycythemia vera and allows a new classification of myeloproliferative diseases. Hematology Am. Soc. Hematol. Educ. Program195–200 (2005).
   PubMedGoogle Scholar
• Dai CH, Krantz SB, Dessypris EN et al. Polycythemia vera. II. Hypersensitivity of bone marrow erythroid, granulocyte–macrophage, and megakaryocyte progenitor cells to interleukin-3 and granulocyte–macrophage colony-stimulating factor. Blood80(4), 891–899 (1992).
   PubMed Web of Science ®Google Scholar
• Dai CH, Krantz SB, Green WF, Gilbert HS. Polycythaemia vera. III. Burst-forming units-erythroid (BFU-E) response to stem cell factor and c-kit receptor expression. Br. J. Haematol.86(1), 12–21 (1994).
   PubMed Web of Science ®Google Scholar
• Dai CH, Krantz SB, Koury ST, Kollar K. Polycythaemia vera. IV. Specific binding of stem cell factor to normal and polycythaemia vera highly purified erythroid progenitor cells. Br. J. Haematol.88(3), 497–505 (1994).
   PubMedGoogle Scholar
• Axelrad AA, Eskinazi D, Correa PN, Amato D. Hypersensitivity of circulating progenitor cells to megakaryocyte growth and development factor (PEG-rHu MGDF) in essential thrombocythemia. Blood96(10), 3310–3321 (2000).
   PubMed Web of Science ®Google Scholar
• Correa PN, Eskinazi D, Axelrad AA. Circulating erythroid progenitors in polycythemia vera are hypersensitive to insulin-like growth factor-1 in vitro: studies in an improved serum-free medium. Blood83(1), 99–112 (1994).
   PubMed Web of Science ®Google Scholar
• Taksin AL, Couedic JP, Dusanter-Fourt I et al. Autonomous megakaryocyte growth in essential thrombocythemia and idiopathic myelofibrosis is not related to a c-mpl mutation or to an autocrine stimulation by Mpl-L. Blood93(1), 125–139 (1999).
   PubMed Web of Science ®Google Scholar
• Tefferi A. Pathogenesis of myelofibrosis with myeloid metaplasia. J. Clin. Oncol.23(33), 8520–8530 (2005).
   PubMed Web of Science ®Google Scholar
• Barosi G, Mesa RA, Thiele J et al. Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the international working group for myelofibrosis research and treatment. Leukemia22, 437–438 (2008).
   PubMed Web of Science ®Google Scholar
• Campbell PJ, Green AR. The myeloproliferative disorders. N. Engl. J. Med.355(23), 2452–2466 (2006).
   PubMed Web of Science ®Google Scholar
• Spivak JL. Diagnosis of the myeloproliferative disorders: resolving phenotypic mimicry. Semin. Hematol.40(1 Suppl. 1), 1–5 (2003).
   PubMed Web of Science ®Google Scholar
• Hehlmann R, Hochhaus A, Baccarani M. Chronic myeloid leukaemia. Lancet370(9584), 342–350 (2007).
   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. Nature434(7037), 1144–1148 (2005).
   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 Cell7(4), 387–397 (2005).
   PubMed Web of Science ®Google Scholar
• Baxter EJ, Scott LM, Campbell PJ et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet365(9464), 1054–1061 (2005).
   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.352(17), 1779–1790 (2005).
   PubMed Web of Science ®Google Scholar
• Levine RL, Pardanani A, Tefferi A, Gilliland DG. Role of JAK2 in the pathogenesis and therapy of myeloproliferative disorders. Nat. Rev. Cancer7(9), 673–683 (2007).
   PubMed Web of Science ®Google Scholar
• Delhommeau F, Pisani DF, James C et al. Oncogenic mechanisms in myeloproliferative disorders. Cell Mol. Life Sci.63(24), 2939–2953 (2006).
   PubMed Web of Science ®Google Scholar
• Dupont S, Masse A, James C et al. The JAK2 V617F mutation triggers erythropoietin hypersensitivity and terminal erythroid amplification in primary cells from patients with polycythemia vera. Blood110, 1013–1021 (2007).
   PubMed Web of Science ®Google Scholar
• Wernig G, Mercher T, Okabe R et al. Expression of Jak2V617F causes a polycythemia vera-like disease with associated myelofibrosis in a murine bone marrow transplant model. Blood107(11), 4274–4281 (2006).
   PubMed Web of Science ®Google Scholar
• Lacout C, Pisani DF, Tulliez M et al. JAK2V617F expression in murine hematopoietic cells leads to MPD mimicking human PV with secondary myelofibrosis. Blood108(5), 1652–1660 (2006).
   PubMed Web of Science ®Google Scholar
• Bumm TG, Elsea C, Corbin AS et al. Characterization of murine JAK2V617F-positive myeloproliferative disease. Cancer Res.66(23), 11156–11165 (2006).
   PubMed Web of Science ®Google Scholar
• Zaleskas VM, Krause DS, Lazarides K et al. Molecular pathogenesis and therapy of polycythemia induced in mice by JAK2 V617F. PLoS ONE1, E18 (2006).
   PubMed Web of Science ®Google Scholar
• Tiedt R, Hao-Shen H, Sobas MA et al. Ratio of mutant JAK2-V617F to wild-type JAK2 determines the MPD phenotypes in transgenic mice. Blood111(8), 3931–3940 (2008).
   PubMed Web of Science ®Google Scholar
• Scott LM, Scott MA, Campbell PJ, Green AR. Progenitors homozygous for the V617F mutation occur in most patients with polycythemia vera, but not essential thrombocythemia. Blood108(7), 2435–2437 (2006).
   PubMed Web of Science ®Google Scholar
• Scott LM, Tong W, Levine RL et al.JAK2 exon 12 mutations in polycythemia vera and idiopathic erythrocytosis. N. Engl. J. Med.356(5), 459–468 (2007).
   PubMed Web of Science ®Google Scholar
• Pardanani AD, Levine RL, Lasho T et al. MPL515 mutations in myeloproliferative and other myeloid disorders: a study of 1182 patients. Blood108(10), 3472–3476 (2006).
   PubMed Web of Science ®Google Scholar
• Pikman Y, Lee BH, Mercher T et al. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med.3(7), E270 (2006).
   PubMed Web of Science ®Google Scholar
• Staerk J, Lacout C, Sato T et al. An amphipathic motif at the transmembrane–cytoplasmic junction prevents autonomous activation of the thrombopoietin receptor. Blood107(5), 1864–1871 (2006).
   PubMed Web of Science ®Google Scholar
• Chaligne R, Tonetti C, Besancenot R et al. New mutations of MPL in primitive myelofibrosis: only the MPL W515 mutations promote a G1/S-phase transition. Leukemia22(8), 1557–1566 (2008).
   PubMed Web of Science ®Google Scholar
• Guglielmelli P, Pancrazzi A, Bergamaschi G et al. Anaemia characterises patients with myelofibrosis harbouring MPL mutation. Br. J. Haematol.137(3), 244–247 (2007).
   PubMed Web of Science ®Google Scholar
• Beer PA, Campbell P, Scott LM et al. MPL mutations in myeloproliferative disorders: analysis of the PT-1 cohort. Blood112(1), 141–149 (2008).
   PubMed Web of Science ®Google Scholar
• Vannucchi AM, Antonioli E, Guglielmelli P et al. Characteristics and clinical correlates of MPL 515W>L/K mutation in essential thrombocythemia. Blood112, 844–847 (2008).
   PubMed Web of Science ®Google Scholar
• Chaligne R, James C, Tonetti C et al. Evidence for MPL W515L/K mutations in hematopoietic stem cells in primitive myelofibrosis. Blood110(10), 3735–3743 (2007).
   PubMed Web of Science ®Google Scholar
• Lasho TL, Pardanani A, McClure RF et al. Concurrent MPL515 and JAK2V617F mutations in myelofibrosis: chronology of clonal emergence and changes in mutant allele burden over time. Br. J. Haematol.135(5), 683–687 (2006).
   PubMed Web of Science ®Google Scholar
• Schnittger S, Bacher U, Haferlach C et al. Characterization of 35 new cases with four different MPLW515 mutations and essential thrombocytosis or primary myelofibrosis. Haematologica94(1), 141–144 (2009).
   PubMedGoogle Scholar
• Tefferi A, Vardiman JW. The diagnostic interface between histology and molecular tests in myeloproliferative disorders. Curr. Opin. Hematol.14(2), 115–122 (2007).
   PubMed Web of Science ®Google Scholar
• Spivak JL, Silver RT. The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis and primary myelofibrosis: an alternative proposal. Blood112(2), 231–239 (2008).
   PubMed Web of Science ®Google Scholar
• McMullin MF, Bareford D, Campbell P et al. Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis. Br. J. Haematol.130(2), 174–195 (2005).
   PubMed Web of Science ®Google Scholar
• McMullin MF, Reilly JT, Campbell P et al. Amendment to the guideline for diagnosis and investigation of polycythaemia/erythrocytosis. Br. J. Haematol.138(6), 821–822 (2007).
   PubMed Web of Science ®Google Scholar
• Vannucchi AM, Antonioli E, Guglielmelli P, Pardanani A, Tefferi A. Clinical correlates of JAK2V617F presence or allele burden in myeloproliferative neoplasms: a critical reappraisal. Leukemia22(7), 1299–1307 (2008).
   PubMed Web of Science ®Google Scholar
• Vannucchi AM, Guglielmelli P. Molecular pathophysiology of Philadelphia-negative myeloproliferative disorders: beyond JAK2 and MPL mutations. Haematologica93(7), 972–976 (2008).
   PubMed Web of Science ®Google Scholar
• Pardanani A, Fridley BL, Lasho TL, Gilliland DG, Tefferi A. Host genetic variation contributes to phenotypic diversity in myeloproliferative disorders. Blood111(5), 2785–2789 (2008).
   PubMedGoogle Scholar
• Nussenzveig RH, Swierczek SI, Jelinek J et al. Polycythemia vera is not initiated by JAK2V617F mutation. Exp. Hematol.35(1), 32–38 (2007).
   PubMed Web of Science ®Google Scholar
• Dameshek W. Some speculations on the myeloproliferative syndromes. Blood6(4), 372–375 (1951).
   PubMed Web of Science ®Google Scholar
• Campbell PJ, Scott LM, Buck G et al. Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study. Lancet366(9501), 1945–1953 (2005).
   PubMed Web of Science ®Google Scholar
• Harrison CN, Green AR. Essential thrombocythaemia. Best Pract. Res. Clin. Haematol.19(3), 439–453 (2006).
   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.23(10), 2224–2232 (2005).
   PubMed Web of Science ®Google Scholar
• Tefferi A. Primary myelofibrosis. Cancer Treat. Res.142, 29–49 (2008).
   PubMedGoogle Scholar
• Landolfi R, Marchioli R, Kutti J et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N. Engl. J. Med.350(2), 114–124 (2004).
   PubMed Web of Science ®Google Scholar
• Harrison CN, Campbell PJ, Buck G et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N. Engl. J. Med.353(1), 33–45 (2005).
   PubMed Web of Science ®Google Scholar
• Gruppo Italiano Policitemia Vera. Polycythemia vera: the natural history of 1213 patients followed for 20 years. Gruppo Italiano Studio Policitemia. Ann. Intern. Med.123(9), 656–664 (1995).
   PubMed Web of Science ®Google Scholar
• De Stefano V, Za T, Rossi E et al. Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments. Haematologica93(3), 372–380 (2008).
   PubMedGoogle Scholar
• Briere JB. Budd–Chiari syndrome and portal vein thrombosis associated with myeloproliferative disorders: diagnosis and management. Semin. Thromb. Hemost.32(3), 208–218 (2006).
   PubMedGoogle 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. Blood111(10), 4922–4929 (2008).
   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. Hepatology44(6), 1528–1534 (2006).
   PubMed Web of Science ®Google 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.5(1), 55–61 (2007).
   PubMed Web of Science ®Google Scholar
• De Stefano V, Fiorini A, Rossi E et al. High prevalence of the JAK2 V617F mutation in patients with extrahepatic portal vein thrombosis. Hepatology45(3), 831–832 (2007).
   PubMedGoogle Scholar
• De Stefano V, Fiorini A, Rossi E et al. Incidence of the JAK2 V617F mutation among patients with splanchnic or cerebral venous thrombosis and without overt chronic myeloproliferative disorders. J. Thromb. Haemost.5(4), 708–714 (2007).
   PubMedGoogle Scholar
• Ugo V, Le Gal G, Lecucq L, Mottier D, Oger E. Prevalence of the JAK2 V617F mutation is low among unselected patients with a first episode of unprovoked venous thromboembolism. J. Thromb. Haemost.6(1), 203–205 (2008).
   PubMedGoogle Scholar
• Pardanani A, Lasho TL, Hussein K et al. JAK2V617F mutation screening as part of the hypercoagulable work-up in the absence of splanchnic venous thrombosis or overt myeloproliferative neoplasm: assessment of value in a series of 664 consecutive patients. Mayo Clin. Proc.83(4), 457–459 (2008).
   PubMedGoogle Scholar
• Guglielmelli P, Fatini C, Lenti M, Bosi A, Vannucchi AM. JAK2V617F mutation screening in patients with retinal vein thrombosis or recurrent fetal loss. Thromb. Res. (2008) (In Press).
   PubMedGoogle Scholar
• Mercier E, Lissalde-Lavigne G, Gris JC. JAK2 V617F mutation in unexplained loss of first pregnancy. N. Engl. J. Med.357(19), 1984–1985 (2007).
   PubMed Web of Science ®Google Scholar
• Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br. J. Haematol.128(3), 275–290 (2005).
   PubMed Web of Science ®Google Scholar
• Michiels JJ, Berneman Z, Van Bockstaele D et al. Clinical and laboratory features, pathobiology of platelet-mediated thrombosis and bleeding complications, and the molecular etiology of essential thrombocythemia and polycythemia vera: therapeutic implications. Semin. Thromb. Hemost.32(3), 174–207 (2006).
   PubMed Web of Science ®Google Scholar
• Spivak J. Daily aspirin – only half the answer. N. Engl. J. Med.350(2), 99–101 (2004).
   PubMedGoogle Scholar
• Di Nisio M, Barbui T, Di Gennaro L et al. The haematocrit and platelet target in polycythemia vera. Br. J. Haematol.136(2), 249–259 (2007).
   PubMed Web of Science ®Google Scholar
• Wolanskyj AP, Lasho TL, Schwager SM et al. JAK2 mutation in essential thrombocythaemia: clinical associations and long-term prognostic relevance. Br. J. Haematol.131(2), 208–213 (2005).
   PubMed Web of Science ®Google Scholar
• Tefferi A, Gangat N, Wolanskyj A. The interaction between leukocytosis and other risk factors for thrombosis in essential thrombocythemia. Blood109(9), 4105 (2007).
   PubMed Web of Science ®Google Scholar
• Carobbio A, Antonioli E, Guglielmelli P et al. Leukocytosis and risk stratification assessment in essential thrombocythemia. J. Clin. Oncol.26(16), 2732–2736 (2008).
   PubMed Web of Science ®Google Scholar
• Carobbio A, Finazzi G, Antonioli E et al. Thrombocytosis and leukocytosis interaction in vascular complications of essential thrombocythemia. Blood112(8), 3135–3137 (2008).
   PubMed Web of Science ®Google Scholar
• Carobbio A, Finazzi G, Guerini V et al. Leukocytosis is a risk factor for thrombosis in essential thrombocythemia: interaction with treatment, standard risk factors, and JAK2 mutation status. Blood109(6), 2310–2313 (2007).
   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. Blood109(6), 2446–2452 (2007).
   PubMed Web of Science ®Google Scholar
• Tefferi A, Gangat N, Wolanskyj AP. Management of extreme thrombocytosis in otherwise low-risk essential thrombocythemia; does number matter? Blood108(7), 2493–2494 (2006).
   PubMedGoogle Scholar
• Cervantes F, Passamonti F, Barosi G. Life expectancy and prognostic factors in the classic BCR/ABL-negative myeloproliferative disorders. Leukemia22(5), 905–914 (2008).
   PubMed Web of Science ®Google Scholar
• Wolanskyj AP, Schwager SM, McClure RF, Larson DR, Tefferi A. Essential thrombocythemia beyond the first decade: life expectancy, long-term complication rates, and prognostic factors. Mayo Clin. Proc.81(2), 159–166 (2006).
   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. Blood105(7), 2664–2670 (2005).
   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. Blood111, 3383–3387 (2008).
   PubMed Web of Science ®Google Scholar
• Murphy S, Peterson P, Iland H, Laszlo J. Experience of the Polycythemia Vera Study Group with essential thrombocythemia: a final report on diagnostic criteria, survival, and leukemic transition by treatment. Semin. Hematol.34(1), 29–39 (1997).
   PubMed Web of Science ®Google Scholar
• Barbui T. The leukemia controversy in myeloproliferative disorders: is it a natural progression of disease, a secondary sequela of therapy, or a combination of both? Semin. Hematol.41(2 Suppl. 3), 15–17 (2004).
   PubMed Web of Science ®Google Scholar
• Tam CS, Nussenzveig RM, Popat U et al. The natural history and treatment outcome of blast phase BCR-ABL negative myeloproliferative neoplasms. Blood112, 1628–1637 (2008).
   PubMed Web of Science ®Google Scholar
• Finazzi G, Barbui T. Risk-adapted therapy in essential thrombocythemia and polycythemia vera. Blood Rev.19(5), 243–252 (2005).
   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.23(2), 132–143 (1986).
   PubMed Web of Science ®Google Scholar
• Harrison CN. Platelets and thrombosis in myeloproliferative diseases. Hematology Am. Soc. Hematol. Educ. Program409–415 (2005).
   PubMedGoogle Scholar
• Antonioli E, Guglielmelli P, Pancrazzi A et al. Clinical implications of the JAK2 V617F mutation in essential thrombocythemia. Leukemia19(10), 1847–1849 (2005).
   PubMed Web of Science ®Google Scholar
• Antonioli E, Guglielmelli P, Poli G et al. Influence of JAK2V617F allele burden on phenotype in essential thrombocythemia. Haematologica93(1), 41–48 (2008).
   PubMed Web of Science ®Google Scholar
• Austin SK, Lambert JR. The JAK2(V617F) mutation and thrombosis. Br. J. Haematol.143(3), 307–320 (2008).
   PubMedGoogle Scholar
• Ziakas PD. Effect of JAK2 V617F on thrombotic risk in patients with essential thrombocythemia: measuring the uncertain. Haematologica93(9), 1412–1414 (2008).
   PubMed Web of Science ®Google Scholar
• Dahabreh IJ, Zoi K, Giannouli S et al. Is JAK2 V617F mutation more than a diagnostic index? A meta-analysis of clinical outcomes in essential thrombocythemia. Leuk. Res.33(1), 67–73 (2008).
   PubMedGoogle Scholar
• Vannucchi AM, Antonioli E, Guglielmelli P et al. Clinical profile of homozygous JAK2V617F mutation in patients with polycythemia vera or essential thrombocythemia. Blood110(3), 840–846 (2007).
   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. Leukemia21, 1952–1959 (2007).
   PubMed Web of Science ®Google Scholar
• Pearson TC, Wetherley-Mein G. Vascular occlusive episodes and venous haematocrit in primary proliferative polycythaemia. Lancet2(8102), 1219–1222 (1978).
   PubMed Web of Science ®Google Scholar
• Streiff MB, Smith B, Spivak JL. The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Study Group: a survey of American Society of Hematology members’ practice patterns. Blood99(4), 1144–1149 (2002).
   PubMed Web of Science ®Google Scholar
• Fruchtman SM, Mack K, Kaplan ME et al. From efficacy to safety: a Polycythemia Vera Study group report on hydroxyurea in patients with polycythemia vera. Semin. Hematol.34(1), 17–23 (1997).
   PubMed Web of Science ®Google Scholar
• Finazzi G, Barbui T. Evidence and expertise in the management of polycythemia vera and essential thrombocythemia. Leukemia22, 1494–1502 (2008).
   PubMed Web of Science ®Google Scholar
• Najean Y, Rain JD. Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood90(9), 3370–3377 (1997).
   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.138(3), 354–358 (2007).
   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.117(10), 755–761 (2004).
   PubMed Web of Science ®Google Scholar
• Weinfeld A, Swolin B, Westin J. Acute leukaemia after hydroxyurea therapy in polycythaemia vera and allied disorders: prospective study of efficacy and leukaemogenicity with therapeutic implications. Eur. J. Haematol.52(3), 134–139 (1994).
   PubMed Web of Science ®Google Scholar
• Berk PD, Goldberg JD, Silverstein MN et al. Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil therapy. N. Engl. J. Med.304(8), 441–447 (1981).
   PubMed Web of Science ®Google Scholar
• Barosi G, Besses C, Birgegard G et al. A unified definition of clinical resistance/intolerance to hydroxyurea in essential thrombocythemia: results of a consensus process by an international working group. Leukemia21(2), 277–280 (2007).
   PubMed Web of Science ®Google Scholar
• Messora C, Bensi L, Vecchi A et al. Cytogenetic conversion in a case of polycythaemia vera treated with interferon-α. Br. J. Haematol.86(2), 402–404 (1994).
   PubMed Web of Science ®Google Scholar
• Liu E, Jelinek J, Pastore YD et al. Discrimination of polycythemias and thrombocytoses by novel, simple, accurate clonality assays and comparison with PRV-1 expression and BFU-E response to erythropoietin. Blood101(8), 3294–3301 (2003).
   PubMed Web of Science ®Google Scholar
• Teofili L, de Stefano V, Iovino MS et al. Disappearance of spontaneous erythroid colonies in patients with myeloproliferative disorders treated by α-interferon. Br. J. Haematol.81(2), 310–311 (1992).
   PubMed Web of Science ®Google Scholar
• Samuelsson J, Mutschler M, Birgegard G et al. Limited effects on JAK2 mutational status after pegylated interferon α2b therapy in polycythemia vera and essential thrombocythemia. Haematologica91(9), 1281–1282 (2006).
   PubMed Web of Science ®Google Scholar
• Steimle C, Lehmann U, Temerinac S et al. Biomarker analysis in polycythemia vera under interferon-α treatment: clonality, EEC, PRV-1, and JAK2 V617F. Ann. Hematol.86(4), 239–244 (2007).
   PubMed Web of Science ®Google Scholar
• Lengfelder E, Berger U, Hehlmann R. Interferon α in the treatment of polycythemia vera. Ann. Hematol.79(3), 103–109 (2000).
   PubMed Web of Science ®Google Scholar
• Silver RT. Long-term effects of the treatment of polycythemia vera with recombinant interferon-α. Cancer107(3), 451–458 (2006).
   PubMed Web of Science ®Google Scholar
• Kiladjian JJ, Cassinat B, Chevret S et al. Pegylated interferon-α2a induces complete hematological and molecular responses with low toxicity in polycythemia vera. Blood112(8), 3065–3072 (2008).
   PubMed Web of Science ®Google Scholar
• Kroger N, Badbaran A, Holler E et al. Monitoring of the JAK2-V617F mutation by highly sensitive quantitative real-time PCR after allogeneic stem cell transplantation in patients with myelofibrosis. Blood109(3), 1316–1321 (2007).
   PubMed Web of Science ®Google Scholar
• Jones AV, Silver RT, Waghorn K et al. Minimal molecular response in polycythemia vera patients treated with imatinib or interferon α. Blood107(8), 3339–3341 (2006).
   PubMed Web of Science ®Google Scholar
• Ricksten A, Palmqvist L, Johansson P, Andreasson B. Rapid decline of JAK2V617F levels during hydroxyurea treatment in patients with polycythemia vera and essential thrombocythemia. Haematologica93(8), 1260–1261 (2008).
   PubMed Web of Science ®Google Scholar
• Girodon F, Schaeffer C, Cleyrat C et al. Frequent reduction or absence of detection of the JAK2-mutated clone in JAK2V617F-positive patients within the first years of hydroxyurea therapy. Haematologica93(8), 1260–1261 (2008).
   PubMedGoogle Scholar
• Landolfi R, Cipriani MC, Novarese L. Thrombosis and bleeding in polycythemia vera and essential thrombocythemia: pathogenetic mechanisms and prevention. Best Pract. Res. Clin. Haematol.19(3), 617–633 (2006).
   PubMed Web of Science ®Google Scholar
• Budde U, Schaefer G, Mueller N et al. Acquired von Willebrand’s disease in the myeloproliferative syndrome. Blood64(5), 981–985 (1984).
   PubMed Web of Science ®Google Scholar
• Castaman G, Lattuada A, Ruggeri M et al. Platelet von Willebrand factor abnormalities in myeloproliferative syndromes. Am. J. Hematol.49(4), 289–293 (1995).
   PubMed Web of Science ®Google Scholar
• Kessler CM. Propensity for hemorrhage and thrombosis in chronic myeloproliferative disorders. Semin. Hematol.41(2 Suppl. 3), 10–14 (2004).
   PubMed Web of Science ®Google Scholar
• Cortelazzo S, Finazzi G, Ruggeri M et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N. Engl. J. Med.332(17), 1132–1136 (1995).
   PubMed Web of Science ®Google Scholar
• Barbui T, Barosi G, Grossi A et al. Practice guidelines for the therapy of essential thrombocythemia. A statement from the Italian Society of Hematology, the Italian Society of Experimental Hematology and the Italian Group for Bone Marrow Transplantation. Haematologica89(2), 215–232 (2004).
   PubMed Web of Science ®Google Scholar
• Lengfelder E, Griesshammer M, Hehlmann R. Interferon-α in the treatment of essential thrombocythemia. Leuk. Lymphoma22(Suppl. 1), 135–142 (1996).
   PubMed Web of Science ®Google Scholar
• Alvarado Y, Cortes J, Verstovsek S et al. Pilot study of pegylated interferon-α2b in patients with essential thrombocythemia. Cancer Chemother. Pharmacol.51(1), 81–86 (2003).
   PubMed Web of Science ®Google Scholar
• Jabbour E, Kantarjian H, Cortes J et al. PEG-IFN-α2b therapy in BCR-ABL-negative myeloproliferative disorders: final result of a Phase 2 study. Cancer110(9), 2012–2018 (2007).
   PubMed Web of Science ®Google Scholar
• Solberg LA Jr, Tefferi A, Oles KJ et al. The effects of anagrelide on human megakaryocytopoiesis. Br. J. Haematol.99(1), 174–180 (1997).
   PubMed Web of Science ®Google Scholar
• Thiele J, Kvasnicka HM, Schmitt-Graeff A. Effects of anagrelide on megakaryopoiesis and platelet production. Semin. Thromb. Hemost.32(4 Pt 2), 352–361 (2006).
   PubMed Web of Science ®Google Scholar
• Fruchtman SM, Petitt RM, Gilbert HS, Fiddler G, Lyne A. Anagrelide: analysis of long-term efficacy, safety and leukemogenic potential in myeloproliferative disorders. Leuk. Res.29(5), 481–491 (2005).
   PubMed Web of Science ®Google Scholar
• Hultdin M, Sundstrom G, Wahlin A et al. Progression of bone marrow fibrosis in patients with essential thrombocythemia and polycythemia vera during anagrelide treatment. Med. Oncol.24(1), 63–70 (2007).
   PubMed Web of Science ®Google Scholar
• Storen EC, Tefferi A. Long-term use of anagrelide in young patients with essential thrombocythemia. Blood97(4), 863–866 (2001).
   PubMed Web of Science ®Google Scholar
• Mazzucconi MG, Redi R, Bernasconi S et al. A long-term study of young patients with essential thrombocythemia treated with anagrelide. Haematologica89(11), 1306–1313 (2004).
   PubMed Web of Science ®Google Scholar
• Birgegard G, Bjorkholm M, Kutti J et al. Adverse effects and benefits of two years of anagrelide treatment for thrombocythemia in chronic myeloproliferative disorders. Haematologica89(5), 520–527 (2004).
   PubMed Web of Science ®Google Scholar
• Steurer M, Gastl G, Jedrzejczak WW et al. Anagrelide for thrombocytosis in myeloproliferative disorders: a prospective study to assess efficacy and adverse event profile. Cancer101(10), 2239–2246 (2004).
   PubMed Web of Science ®Google Scholar
• Birgegard G. Long-term management of thrombocytosis in essential thrombocythaemia. Ann. Hematol. (2008).
   PubMedGoogle Scholar
• Barbui T, Finazzi G. Myeloproliferative disease in pregnancy and other management issues. Hematology Am. Soc. Hematol. Educ. Program246–252 (2006).
   PubMedGoogle Scholar
• Griesshammer M, Struve S, Barbui T. Management of Philadelphia negative chronic myeloproliferative disorders in pregnancy. Blood Rev.22(5), 235–245 (2008).
   PubMed Web of Science ®Google Scholar
• Harrison C. Pregnancy and its management in the Philadelphia negative myeloproliferative diseases. Br. J. Haematol.129(3), 293–306 (2005).
   PubMed Web of Science ®Google Scholar
• Passamonti F, Randi ML, Rumi E et al. Increased risk of pregnancy complications in patients with essential thrombocythemia carrying the JAK2 (617V>F) mutation. Blood110(2), 485–489 (2007).
   PubMed Web of Science ®Google Scholar
• Tefferi A. JAK and MPL mutations in myeloid malignancies. Leuk. Lymphoma49(3), 388–397 (2008).
   PubMed Web of Science ®Google Scholar
• Druker BJ, Guilhot F, O’Brien SG et al. Five-year follow-up of patients receiving imatinib for chronic myeloid leukemia. N. Engl. J. Med.355(23), 2408–2417 (2006).
   PubMed Web of Science ®Google Scholar
• Pardanani A. JAK2 inhibitor therapy in myeloproliferative disorders: rationale, preclinical studies and ongoing clinical trials. Leukemia22(1), 23–30 (2008).
   PubMed Web of Science ®Google Scholar
• Pardanani A, Hood J, Lasho T et al. TG101209, a small molecule JAK2-selective kinase inhibitor potently inhibits myeloproliferative disorder-associated JAK2V617F and MPLW515L/K mutations. Leukemia21(8), 1658–1668 (2007).
   PubMed Web of Science ®Google Scholar
• Lasho TL, Tefferi A, Hood JD et al. TG101348, a JAK2-selective antagonist, inhibits primary hematopoietic cells derived from myeloproliferative disorder patients with JAK2V617F, MPLW515K or JAK2 exon 12 mutations as well as mutation negative patients. Leukemia22(9), 1790–1792 (2008).
   PubMed Web of Science ®Google Scholar
• Fridman J, Nussenzveig R, Liu P et al. Discovery and preclinical characterization of INCB018424, a selective JAK2 inhibitor for the treatment of myeloproliferative disorders. ASH Annual Meeting Abstracts110(11), 3538 (2007).
   Google Scholar
• Verstovsek S, Pardanani AD, Shah NP et al. A Phase I study of XL019, a selective JAK2 inhibitor, in patients with primary myelofibrosis and post-polycythemia vera/essential thrombocythemia myelofibrosis. ASH Annual Meeting Abstracts110(11), 553 (2007).
   Google Scholar
• Mesa RA, Camoriano JK, Geyer SM et al. A Phase II trial of tipifarnib in myelofibrosis: primary, post-polycythemia vera and post-essential thrombocythemia. Leukemia21(9), 1964–1970 (2007).
   PubMed Web of Science ®Google Scholar
• Guerini V, Barbui V, Spinelli O et al. The histone deacetylase inhibitor ITF2357 selectively targets cells bearing mutated JAK2(V617F). Leukemia22(4), 740–747 (2008).
   PubMed Web of Science ®Google Scholar
• Hexner EO, Serdikoff C, Jan M et al. Lestaurtinib (CEP701) is a JAK2 inhibitor that suppresses JAK2/STAT5 signaling and the proliferation of primary erythroid cells from patients with myeloproliferative disorders. Blood111, 5663–5671 (2008).
   PubMed Web of Science ®Google Scholar
• Geron I, Abrahamsson AE, Barroga CF et al. Selective inhibition of JAK2-driven erythroid differentiation of polycythemia vera progenitors. Cancer Cell13(4), 321–330 (2008).
   PubMed Web of Science ®Google Scholar