Polycythemia vera

References

Key articles

Adamson JW, Fialkow PJ, Murphy S, et al. Polycythemia vera: stem-cell and probable clonal origin of the disease. N Engl J Med. 1976 Oct 21;295(17):913-6. Abstract

James C, Ugo V, Le Couedic JP, et al. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature. 2005 Apr 28;434(7037):1144-8. Abstract

Landolfi R, Marchioli R, Kutti J, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med. 2004 Jan 8;350(2):114-24.Full text  Abstract

Tefferi A, Rumi E, Finazzi G, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013 Sep;27(9):1874-81.Full text  Abstract

Marchioli R, Finazzi G, Specchia G, et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013 Jan 3;368(1):22-33.Full text  Abstract

Vannucchi AM, Kiladjian JJ, Griesshammer M, et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015 Jan 29;372(5):426-35. Abstract

Reference articles

1. Adamson JW, Fialkow PJ, Murphy S, et al. Polycythemia vera: stem-cell and probable clonal origin of the disease. N Engl J Med. 1976 Oct 21;295(17):913-6. Abstract

2. Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br J Haematol. 2005 Feb;128(3):275-90. Abstract

3. James C, Ugo V, Le Couedic JP, et al. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature. 2005 Apr 28;434(7037):1144-8. Abstract

4. Kralovics R, Passamonti F, Buser AS, et al. A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med. 2005 Apr 28;352(17):1779-90.Full text  Abstract

5. Baxter EJ, Scott LM, Campbell PJ, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet. 2005 Mar 19-25;365(9464):1054-61. Abstract

6. Levine RL, Wadleigh M, Cools J, et al. Activating mutation in the tyrosine kinase JAK2 in polycythaemia vera, essential thrombocythemia and myeloid metaplasia with myelofibrosis. Cancer Cell. 2005 Apr;7(4):387-97. Abstract

7. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19;127(20):2391-405.Full text  Abstract

8. Finazzi G, Barbui T. How I treat patients with polycythemia vera. Blood. 2007 Jun 15;109(12):5104-11.Full text  Abstract

9. Vannucchi AM. How I treat polycythemia vera. Blood. 2014 Nov 20;124(22):3212-20.Full text  Abstract

10. McMullin MF, Harrison CN, Ali S, et al. A guideline for the diagnosis and management of polycythaemia vera: a British Society for Haematology guideline. Br J Haematol. 2019 Jan;184(2):176-91. Abstract

11. Johansson P. Epidemiology of the myeloproliferative disorders polycythemia vera and essential thrombocythemia. Semin Thromb Hemost. 2006 Apr;32(3):171-3. Abstract

12. Ania BJ, Suman VJ, Sobell JL, et al. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989. Am J Hematol. 1994 Oct;47(2):89-93. Abstract

13. Mehta J, Wang H, Iqbal SU, et al. Epidemiology of myeloproliferative neoplasms in the United States. Leuk Lymphoma. 2014 Mar;55(3):595-600. Abstract

14. 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 Apr 1;23(10):2224-32.Full text  Abstract

15. Landolfi R, Marchioli R, Kutti J, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med. 2004 Jan 8;350(2):114-24.Full text  Abstract

16. Vannucchi AM. How I treat polycythemia vera. Blood. 2014 Nov 20;124(22):3212-20.Full text  Abstract

17. Patel RK, Lea NC, Heneghan MA, et al. Prevalence of the activating JAK2 tyrosine kinase mutation V617F in the Budd-Chiari Syndrome. Gastroenterology. 2006 Jun;130(7):2031-8. Abstract

18. Barbui T, Thiele J, Carobbio A, et al. Masked polycythemia vera diagnosed according to WHO and BCSH classification. Am J Hematol. 2014;89:199-202.Full text  Abstract

19. Barbui T, Thiele J, Gisslinger H, et al. Masked polycythemia vera (mPV): results of an international study. Am J Hematol. 2014 Jan;89(1):52-4.Full text  Abstract

20. Lussana F, Carobbio A, Randi ML, et al. A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythaemia vera. Br J Haematol. 2014 Nov;167(4):541-6.Full text  Abstract

21. Barbui T, Thiele J, Carobbio A, et al. Discriminating between essential thrombocythemia and masked polycythemia vera in JAK2 mutated patients. Am J Hematol. 2014 Jun;89(6):588-90.Full text  Abstract

22. Cario H, McMullin MF, Pahl HL. Clinical and hematological presentation of children and adolescents with polycythemia vera. Ann Hematol. 2009 Aug;88(8):713-9. Abstract

23. Moulard O, Mehta J, Fryzek J, et al. Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union. Eur J Haematol. 2014 Apr;92(4):289-97.Full text  Abstract

24. Tefferi A, Rumi E, Finazzi G, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013 Sep;27(9):1874-81.Full text  Abstract

25. Modan B, Kallner H, Zemer D, et al. A note on the increased risk of polycythemia vera in Jews. Blood. 1971 Feb;37(2):172-6.Full text  Abstract

26. Pardanani A, Lasho TL, Finke C, et al. Prevalence and clinicopathologic correlates of JAK2 exon 12 mutations in JAK2V617F-negative polycythemia vera. Leukemia. 2007 Sep;21(9):1960-3. Abstract

27. Lasho TL, Pardanani A, Tefferi A. LNK mutations in JAK2 mutation-negative erythrocytosis. N Engl J Med. 2010 Sep 16;363(12):1189-90.Full text  Abstract

28. Jang MA, Choi CW. Recent insights regarding the molecular basis of myeloproliferative neoplasms. Korean J Intern Med. 2020 Jan;35(1):1-11.Full text  Abstract

29. Levine RL, Loriaux M, Huntly BJ, et al. The JAK2V617F activating mutation occurs in chronic myelomonocytic leukemia and acute myeloid leukemia, but not in acute lymphoblastic leukemia or chronic lymphocytic leukemia. Blood. 2005 Nov 15;106(10):3377-9.Full text  Abstract

30. Vainchenker W, Delhommeau F, Constantinescu SN, et al. New mutations and pathogenesis of myeloproliferative neoplasms. Blood. 2011 Aug 18;118(7):1723-35.Full text  Abstract

31. Olcaydu D, Harutyunyan A, Jäger R, et al. A common JAK2 haplotype confers susceptibility to myeloproliferative neoplasms. Nat Genet. 2009 Apr;41(4):450-4. Abstract

32. Kilpivaara O, Mukherjee S, Schram AM, et al. A germline JAK2 SNP is associated with predisposition to the development of JAK2(V617F)-positive myeloproliferative neoplasms. Nat Genet. 2009 Apr;41(4):455-9. Abstract

33. Jones AV, Chase A, Silver RT, et al. JAK2 haplotype is a major risk factor for the development of myeloproliferative neoplasms. Nat Genet. 2009 Apr;41(4):446-9. Abstract

34. Tefferi A, Lasho TL, Guglielmelli P, et al. Targeted deep sequencing in polycythemia vera and essential thrombocythemia. Blood Adv. 2016 Nov 22;1(1):21-30.Full text  Abstract

35. Boddu P, Mansurov A, Masarova L, et al. Non-driver mutations and gene sequence variants identified by targeted deep sequencing in polycythemia vera and essential thrombocythemia. Blood. 130;(suppl 1):4183.Full text

36. Genovese G, Kähler AK, Handsaker RE, et al. Clonal hematopoiesis and blood-cancer risk inferred from blood DNA sequence. N Engl J Med. 2014 Dec 25;371(26):2477-87.Full text  Abstract

37. Jaiswal S, Fontanillas P, Flannick J, et al. Age-related clonal hematopoiesis associated with adverse outcomes. N Engl J Med. 2014 Dec 25;371(26):2488-98.Full text  Abstract

38. Segura-Díaz A, Stuckey R, Florido Y, et al. Thrombotic risk detection in patients with polycythemia vera: the predictive role of DNMT3A/TET2/ASXL1 mutations. Cancers (Basel). 2020 Apr 10;12(4):934.Full text  Abstract

39. Ortmann CA, Kent DG, Nangalia J, et al. Effect of mutation order on myeloproliferative neoplasms. N Engl J Med. 2015 Feb 12;372(7):601-12.Full text  Abstract

40. Nangalia J, Nice FL, Wedge DC, et al. DNMT3A mutations occur early or late in patients with myeloproliferative neoplasms and mutation order influences phenotype. Haematologica. 2015 Nov;100(11):e438-42.Full text  Abstract

41. 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 Sep 15;112(6):2199-204.Full text  Abstract

42. Vannucchi AM, Antonioli E, Guglielmelli P, et al. Clinical profile of homozygous JAK2 617V>F mutation in patients with polycythemia vera or essential thrombocythemia. Blood. 2007 Aug 1;110(3):840-6.Full text  Abstract

43. Tefferi A, Lasho TL, Schwager SM, et al. The clinical phenotype of wild-type, heterozygous, and homozygous JAK2V617F in polycythemia vera. Cancer. 2006 Feb 1;106(3):631-5.Full text  Abstract

44. 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 Sep;24(9):1574-9.Full text  Abstract

45. Pearson TC, Wetherlye-Mein G. Vascular occlusive episodes and venous haematocrit in primary proliferative polycythaemia. Lancet. 1978 Dec 9;2(8102):1219-22. Abstract

46. Spivak JL. Polycythemia vera: myths, mechanisms, and management. Blood. 2002 Dec 15;100(13):4272-90.Full text  Abstract

47. Marchioli R, Finazzi G, Specchia G, et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013 Jan 3;368(1):22-33.Full text  Abstract

48. Landolfi R, Di Gennaro L, Barbui T, et al; European Collaboration on Low-Dose Aspirin in Polycythemia Vera (ECLAP). Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood. 2007 Mar 15;109(6):2446-52.Full text  Abstract

49. Tefferi A, Elliott M. Thrombosis in myeloproliferative disorders: prevalence, prognostic factors, and the role of leukocytes and JAK2V617F. Semin Thromb Hemost. 2007 Jun;33(4):313-20. Abstract

50. Wolach O, Sellar RS, Martinod K, et al. Increased neutrophil extracellular trap formation promotes thrombosis in myeloproliferative neoplasms. Sci Transl Med. 2018 Apr 11;10(436):eaan8292. Abstract

51. Ronner L, Podoltsev N, Gotlib J, et al. Persistent leukocytosis in polycythemia vera is associated with disease evolution but not thrombosis. Blood. 2020 May 7;135(19):1696-703.Full text  Abstract

52. Di Nisio M, Barbui T, Di Gennaro L, et al; European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Investigators. The haematocrit and platelet target in polycythemia vera. Br J Haematol. 2007 Jan;136(2):249-59.Full text  Abstract

53. Carobbio A, Thiele J, Passamonti F, et al. Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients. Blood. 2011 Jun 2;117(22):5857-9.Full text  Abstract

54. Carobbio A, Finazzi G, Antonioli E, et al. Thrombocytosis and leukocytosis interaction in vascular complications of essential thrombocythemia. Blood. 2008 Oct 15;112(8):3135-7.Full text  Abstract

55. Spivak JL, Considine M, Williams DM, et al. Two clinical phenotypes in polycythemia vera. N Engl J Med. 2014 Aug 28;371(9):808-17.Full text  Abstract

56. Fowles JS, How J, Allen MJ, et al. Young versus old age at diagnosis confers distinct genomic profiles in patients with polycythemia vera. Leukemia. 2019 Jun;33(6):1522-26. Abstract

57. Rumi E, Passamonti F, Pietra D, et al. JAK2 (V617F) as an acquired somatic mutation and a secondary genetic event associated with disease progression in familial myeloproliferative disorders. Cancer. 2006 Nov 1;107(9):2206-11.Full text  Abstract

58. Smalberg JH, Arends LR, Valla DC, et al. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood. 2012 Dec 13;120(25):4921-8.Full text  Abstract

59. Mesa RA, Niblack J, Wadleigh M, et al. The burden of fatigue and quality of life in myeloproliferative disorders (MPDs): an international Internet-based survey of 1179 MPD patients. Cancer. 2007 Jan 1;109(1):68-76.Full text  Abstract

60. Anawalt BD. Diagnosis and management of anabolic androgenic steroid use. J Clin Endocrinol Metab. 2019 Jul 1;104(7):2490-500.Full text  Abstract

61. Ohlander SJ, Varghese B, Pastuszak AW. Erythrocytosis following testosterone therapy. Sex Med Rev. 2018 Jan;6(1):77-85.Full text  Abstract

62. Cross NCP, Godfrey AL, Cargo C, et al. The use of genetic tests to diagnose and manage patients with myeloproliferative and myeloproliferative/myelodysplastic neoplasms, and related disorders. Br J Haematol. 2021 Nov;195(3):338-351.Full text  Abstract

63. Jaiswal S, Natarajan P, Silver AJ, et al. Clonal hematopoiesis and risk of atherosclerotic cardiovascular disease. N Engl J Med. 2017 Jul 13;377(2):111-21.Full text  Abstract

64. 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. Blood. 2007 Aug 15;110(4):1092-7.Full text  Abstract

65. Tefferi A, Thiele J, Vannucchi AM, et al. An overview on CALR and CSF3R mutations and a proposal for revision of WHO diagnostic criteria for myeloproliferative neoplasms. Leukemia. 2014 Jul;28(7):1407-13. Abstract

66. Barbui T, Thiele J, Passamonti F, et al. Initial bone marrow reticulin fibrosis in polycythemia vera exerts an impact on clinical outcome. Blood. 2012 Mar 8;119(10):2239-41.Full text  Abstract

67. Cao M, Olsen RJ, Zu Y. Polycythemia vera: new clinicopathologic perspectives. Arch Pathol Lab Med. 2006 Aug;130(8):1126-32.Full text  Abstract

68. Tefferi A, Guglielmelli P, Lasho TL, et al. Mutation-enhanced international prognostic systems for essential thrombocythaemia and polycythaemia vera. Br J Haematol. 2020 Apr;189(2):291-302.Full text  Abstract

69. Scott LM, Tong W, Levine RL, et al. JAK2 exon 12 mutations in polycythemia vera and idiopathic erythrocytosis. N Engl J Med. 2007 Feb 1;356(5):459-68.Full text  Abstract

70. Pikman Y, Lee BH, Mercher T, et al. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med. 2006 Jul;3(7):e270.Full text  Abstract

71. Pardanani AD, Levine RL, Lasho T, et al. MPL515 mutations in myeloproliferative and other myeloid disorders: a study of 1182 patients. Blood. 2006 Nov 15;108(10):3472-6.Full text  Abstract

72. Nangalia J, Massie CE, Baxter EJ, et al. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N Engl J Med. 2013 Dec 19;369(25):2391-2405.Full text  Abstract

73. Klampfl T, Gisslinger H, Harutyunyan AS, et al. Somatic mutations of calreticulin in myeloproliferative neoplasms. N Engl J Med. 2013 Dec 19;369(25):2379-90.Full text  Abstract

74. Broséus J, Park JH, Carillo S, et al. Presence of calreticulin mutations in JAK2-negative polycythemia vera. Blood. 2014 Dec 18;124(26):3964-6.Full text  Abstract

75. Shih LY, Lee CT. Identification of masked polycythemia vera from patients with idiopathic marked thrombocytosis by endogenous erythroid colony assay. Blood. 1994 Feb 1;83(3):744-8.Full text  Abstract

76. Beutler E. Polycythemia. In: Beutler E, Lichtman MA, Coller BS, et al, eds. Williams hematology. 6th ed. New York: McGraw-Hill; 2001:689-701.

77. Mesa R, Boccia RV, Grunwald MR, et al. Patient-reported outcomes data from REVEAL at the time of enrollment (baseline): a prospective observational study of patients with polycythemia vera in the United States. Clin Lymphoma Myeloma Leuk. 2018 Sep;18(9):590-6.Full text  Abstract

78. Saini KS, Patnaik MM, Tefferi A. Polycythemia vera-associated pruritus and its management. Eur J Clin Invest. 2010 Sep;40(9):828-34. Abstract

79. Vannucchi AM, Kiladjian JJ, Griesshammer M, et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015 Jan 29;372(5):426-35. Abstract

80. Gangat N, Strand JJ, Lasho TL, et al. Pruritus in polycythemia vera is associated with a lower risk of arterial thrombosis. Am J Hematol. 2008 Jun;83(6):451-3.Full text  Abstract

81. Gangat N, Strand J, Li CY, et al. Leucocytosis in polycythaemia vera predicts both inferior survival and leukaemic transformation. Br J Haematol. 2007 Aug;138(3):354-8. Abstract

82. Vannucchi AM, Antonioli E, Guglielmelli P, et al. Prospective identification of high-risk polycythemia vera patients based on JAK2(V617F) allele burden. Leukemia. 2007 Sep;21(9):1952-9. Abstract

83. 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 Feb;35(2):177-82.Full text  Abstract

84. Nussenzveig RH, Swierczek SI, Jelinek J, et al. Polycythemia vera is not initiated by JAK2V617F mutation. Exp Hematol. 2007 Jan;35(1):32-8. Abstract

85. Barbui T, Thiele J, Kvasnicka HM, et al. Essential thrombocythemia with high hemoglobin levels according to the revised WHO classification. Leukemia. 2014 Oct;28(10):2092-4. Abstract

86. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms [internet publication].Full text

87. Barbui T, Tefferi A, Vannucchi AM, et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia. 2018 May;32(5):1057-69. Abstract

88. Podoltsev NA, Zhu M, Zeidan AM, et al. The impact of phlebotomy and hydroxyurea on survival and risk of thrombosis among older patients with polycythemia vera. Blood Adv. 2018 Oct 23;2(20):2681-90.Full text  Abstract

89. Tartaglia AP, Goldberg JD, Berk PD, et al. Adverse effects of antiaggregating platelet therapy in the treatment of polycythemia vera. Semin Hematol. 1986 Jul;23(3):172-6. Abstract

90. Tefferi A, Pardanani A. Essential thrombocythemia. N Engl J Med. 2019 Nov 28;381(22):2135-44. Abstract

91. Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol. 2017 Jan;92(1):94-108. Abstract

92. Rocca B, Tosetto A, Betti S, et al. A randomized double-blind trial of 3 aspirin regimens to optimize antiplatelet therapy in essential thrombocythemia. Blood. 2020 Jul 9;136(2):171-82.Full text  Abstract

93. Mesa RA, Jamieson C, Bhatia R, et al. NCCN guidelines insights: myeloproliferative neoplasms, version 2, 2018. J Natl Compr Canc Netw. 2017 Oct;15(10):1193-207.Full text  Abstract

94. Najean Y, Rain JD for the French Polycythemia Vera Study Group. Treatment of polycthemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood. 1997 Nov 1;90(9):3370-7.Full text  Abstract

95. Cortelazzo S, Finazzi G, Ruggeri M, et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995 Apr 27;332(17):1132-6.Full text  Abstract

96. Harrison CN, Campbell PJ, Buck G, et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005 Jul 7;353(1):33-45.Full text  Abstract

97. Barbui T, Vannucchi AM, Finazzi G, et al. A reappraisal of the benefit-risk profile of hydroxyurea in polycythemia vera: a propensity-matched study. Am J Hematol. 2017 Nov;92(11):1131-36.Full text  Abstract

98. De Stefano V, Rossi E, Carobbio A, et al. Hydroxyurea prevents arterial and late venous thrombotic recurrences in patients with myeloproliferative neoplasms but fails in the splanchnic venous district. Pooled analysis of 1500 cases. Blood Cancer J. 2018 Nov 12;8(11):112.Full text  Abstract

99. 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 Apr 1;105(7):2664-70.Full text  Abstract

100. Tefferi A, Vannucchi AM, Barbui T. Essential thrombocythemia treatment algorithm 2018. Blood Cancer J. 2018 Jan 10;8(1):2.Full text  Abstract

101. Alvarez-Larrán A, Pereira A, Cervantes F, et al. Assessment and prognostic value of the European LeukemiaNet criteria for clinicohematologic response, resistance, and intolerance to hydroxyurea in polycythemia vera. Blood. 2012 Feb 9;119(6):1363-9.Full text  Abstract

102. Barosi G, Birgegard G, Finazzi G, et al. A unified definition of clinical resistance and intolerance to hydroxycarbamide in polycythaemia vera and primary myelofibrosis: results of a European LeukemiaNet (ELN) consensus process. Br J Haematol. 2010 Mar;148(6):961-3. Abstract

103. Silver RT. Treatment of polycythemia vera. Semin Thromb Hemost. 2006 Jun;32(4 Pt 2):437-42. Abstract

104. Kiladjian JJ, Cassinat B, Turlure P, et al. High molecular response rate of polycythemia vera patients treated with pegylated interferon alpha-2a. Blood. 2006 Sep 15;108(6):2037-40.Full text  Abstract

105. Barosi G, Mesa R, Finazzi G, et al. Revised response criteria for polycythemia vera and essential thrombocythemia: an ELN and IWG-MRT consensus project. Blood. 2013 Jun 6;121(23):4778-81.Full text  Abstract

106. Vannucchi AM, Barbui T, Cervantes F, et al; ESMO Guidelines Committee. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26 Suppl 5:v85-99.Full text  Abstract

107. Quintás-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 Nov 10;27(32):5418-24.Full text  Abstract

108. Masarova L, Patel KP, Newberry KJ, et al. Pegylated interferon alfa-2a in patients with essential thrombocythaemia or polycythaemia vera: a post-hoc, median 83 month follow-up of an open-label, phase 2 trial. Lancet Haematol. 2017 Apr;4(4):e165-75.Full text  Abstract

109. Yacoub A, Mascarenhas J, Kosiorek H, et al. Pegylated interferon alfa-2a for polycythemia vera or essential thrombocythemia resistant or intolerant to hydroxyurea. Blood. 2019 Oct 31;134(18):1498-509.Full text  Abstract

110. Mascarenhas J, Kosiorek HE, Prchal JT, et al. Results of the Myeloproliferative Neoplasms - Research Consortium (MPN-RC) 112 randomized trial of pegylated interferon alfa-2a (PEG) versus hydroxyurea (HU) therapy for the treatment of high risk polycythemia vera (PV) and high risk essential thrombocythemia (ET). Blood. 2018 Nov 29;132(supp 1):577.Full text

111. Kiladjian JJ, Massé A, Cassinat B, et al; French Intergroup of Myeloproliferative Neoplasms (FIM). Clonal analysis of erythroid progenitors suggests that pegylated interferon alpha-2a treatment targets JAK2V617F clones without affecting TET2 mutant cells. Leukemia. 2010 Aug;24(8):1519-23. Abstract

112. Quintás-Cardama A, Abdel-Wahab O, Manshouri T, et al. Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a. Blood. 2013 Aug 8;122(6):893-901.Full text  Abstract

113. Passamonti F, Griesshammer M, Palandri F, et al. Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): a randomised, open-label, phase 3b study. Lancet Oncol. 2017 Jan;18(1):88-99. Abstract

114. Kiladjian JJ, Zachee P, Hino M, et al. Long-term efficacy and safety of ruxolitinib versus best available therapy in polycythaemia vera (RESPONSE): 5-year follow up of a phase 3 study. Lancet Haematol. 2020 Mar;7(3):e226-37. Abstract

115. Masciulli A, Ferrari A, Carobbio A, et al. Ruxolitinib for the prevention of thrombosis in polycythemia vera: a systematic review and meta-analysis. Blood Adv. 2020 Jan 28;4(2):380-6.Full text  Abstract

116. Mesa R, Vannucchi AM, Yacoub A, et al. The efficacy and safety of continued hydroxycarbamide therapy versus switching to ruxolitinib in patients with polycythaemia vera: a randomized, double-blind, double-dummy, symptom study (RELIEF). Br J Haematol. 2017 Jan;176(1):76-85.Full text  Abstract

117. Pieri L, Pancrazzi A, Pacilli A, et al. JAK2V617F complete molecular remission in polycythemia vera/essential thrombocythemia patients treated with ruxolitinib. Blood. 2015 May 21;125(21):3352-3.Full text  Abstract

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