Polycythemia vera

References

Key articles

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

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

Tefferi A, Barbui T. Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023 Sep;98(9):1465-87.Full text  Abstract

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

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

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.Full text  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 RP, et al. International consensus classification of myeloid neoplasms and acute leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022 Sep 15;140(11):1200-28.Full text  Abstract

8. Khoury JD, Solary E, Abla O, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms. Leukemia. 2022 Jul;36(7):1703-19.Full text  Abstract

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

10. 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

11. Verstovsek S, Yu J, Scherber RM, et al. Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States. Leuk Lymphoma. 2022 Mar;63(3):694-702.Full text  Abstract

12. Grunwald MR, Stein BL, Boccia RV, et al. Clinical and disease characteristics from REVEAL at time of enrollment (baseline): prospective observational study of patients with polycythemia vera in the United States. Clin Lymphoma Myeloma Leuk. 2018 Dec;18(12):788-95.Full text  Abstract

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

14. 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

15. 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

16. 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

17. 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

18. 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

19. 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

20. Haematological Malignancy Research Network (HMRN). Cancer factsheets: polycythaemia vera. May 2024 [internet publication].​Full text

21. 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

22. Chaiter Y, Brenner B, Aghai E, et al. High incidence of myeloproliferative disorders in Ashkenazi Jews in northern Israel. Leuk Lymphoma. 1992 Jun;7(3):251-5. Abstract

23. Le M, Ghazawi FM, Rahme E, et al. Identification of significant geographic clustering of polycythemia vera cases in Montreal, Canada. Cancer. 2019 Nov 15;125(22):3953-9.Full text  Abstract

24. Ma X, Vanasse G, Cartmel B, et al. Prevalence of polycythemia vera and essential thrombocythemia. Am J Hematol. 2008 May;83(5):359-62. Abstract

25. Titmarsh GJ, Duncombe AS, McMullin MF, et al. How common are myeloproliferative neoplasms? A systematic review and meta-analysis. Am J Hematol. 2014 Jun;89(6):581-7.Full text  Abstract

26. 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

27. Ishida H, Miyajima Y, Hyakuna N, et al. Clinical features of children with polycythemia vera, essential thrombocythemia, and primary myelofibrosis in Japan: a retrospective nationwide survey. EJHaem. 2020 Jul;1(1):86-93.Full text  Abstract

28. Scott LM. The JAK2 exon 12 mutations: a comprehensive review. Am J Hematol. 2011 Aug;86(8):668-76.Full text  Abstract

29. 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

30. 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

31. 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

32. 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

33. 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

34. 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

35. 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

36. 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

37. 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

38. 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

39. 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

40. 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

41. 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

42. 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

43. 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

44. 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

45. Williams N, Lee J, Mitchell E, et al. Life histories of myeloproliferative neoplasms inferred from phylogenies. Nature. 2022 Feb;602(7895):162-8. Abstract

46. Allahverdi N, Yassin M, Ibrahim M. Environmental factors, lifestyle risk factors, and host characteristics associated with Philadelphia negative myeloproliferative neoplasm: a systematic review. Cancer Control. 2021 Jan-Dec;28:10732748211046802.Full text  Abstract

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

48. Guglielmelli P, Loscocco GG, Mannarelli C, et al. JAK2V617F variant allele frequency >50% identifies patients with polycythemia vera at high risk for venous thrombosis. Blood Cancer J. 2021 Dec 11;11(12):199.Full text  Abstract

49. Chen CC, Chen JL, Lin AJ, et al. Association of JAK2V617F allele burden and clinical correlates in polycythemia vera: a systematic review and meta-analysis. Ann Hematol. 2024 Apr 23.Full text  Abstract

50. Moliterno AR, Kaizer H, Reeves BN. JAK2 V617F allele burden in polycythemia vera: burden of proof. Blood. 2023 Apr 20;141(16):1934-42.Full text  Abstract

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

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

53. 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

54. 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

55. 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

56. 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

57. 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

58. 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

59. 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

60. 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

61. 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

62. 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

63. 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

64. 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

65. 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

66. 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

67. Spivak JL. How I treat polycythemia vera. Blood. 2019 Jul 25;134(4):341-52.Full text  Abstract

68. 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

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

70. 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

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

72. Zhang J, DeMeo DL, Silverman EK, et al. Secondary polycythemia in chronic obstructive pulmonary disease: prevalence and risk factors. BMC Pulm Med. 2021 Jul 14;21(1):235.Full text  Abstract

73. Fawzy A, Woo H, Balasubramanian A, et al. Polycythemia is associated with lower incidence of severe COPD exacerbations in the SPIROMICS study. Chronic Obstr Pulm Dis. 2021 Jul 28;8(3):326-35.Full text  Abstract

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

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

76. 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

77. 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

78. 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

79. 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

80. 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

81. 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

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

83. 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

84. 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

85. 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

86. 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

87. 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

88. 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

89. Shimoyama T, Yamaguchi H, Kimura K, et al. Clinical characteristics and management of headache in patients with myeloproliferative neoplasms. Front Neurol. 2022 Dec 6;13:1051093.Full text  Abstract

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

91. 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

92. 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

93. 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

94. Andriani A, Latagliata R, Anaclerico B, et al. Spleen enlargement is a risk factor for thrombosis in essential thrombocythemia: evaluation on 1,297 patients. Am J Hematol. 2016 Mar;91(3):318-21.Full text  Abstract

95. Accurso V, Santoro M, Raso S, et al. Splenomegaly impacts prognosis in essential thrombocythemia and polycythemia vera: a single center study. Hematol Rep. 2019 Nov 29;11(4):8281.Full text  Abstract

96. 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

97. Tefferi A, Barbui T. Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023 Sep;98(9):1465-87.Full text  Abstract

98. 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

99. Emanuel RM, Dueck AC, Geyer HL, et al. Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. J Clin Oncol. 2012 Nov 20;30(33):4098-103.Full text  Abstract

100. Barosi G, Mesa RA, Thiele J, et al; International Working Group for Myelofibrosis Research and Treatment (IWG-MRT). 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. Leukemia. 2008 Feb;22(2):437-8. Abstract

101. Thiele J, Kvasnicka HM, Facchetti F, et al. European consensus on grading bone marrow fibrosis and assessment of cellularity. Haematologica. 2005 Aug;90(8):1128-32.Full text  Abstract

102. Manoharan A, Horsley R, Pitney WR. The reticulin content of bone marrow in acute leukaemia in adults. Br J Haematol. 1979 Oct;43(2):185-90. Abstract

103. 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

104. 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

105. 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

106. 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

107. 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

108. Barbui T, Passamonti F, Accorsi P, et al. Evidence- and consensus-based recommendations for phlebotomy in polycythemia vera. Leukemia. 2018 Sep;32(9):2077-81. Abstract

109. Barbui T, Vannucchi AM, De Stefano V, et al. Ropeginterferon alfa-2b versus phlebotomy in low-risk patients with polycythaemia vera (Low-PV study): a multicentre, randomised phase 2 trial. Lancet Haematol. 2021 Mar;8(3):e175-e184. Abstract

110. Barbui T, Vannucchi AM, De Stefano V, et al. Ropeginterferon versus standard therapy for low-risk patients with polycythemia vera. NEJM Evid. 2023 Jun;2(6):EVIDoa2200335.Full text  Abstract

111. Vachhani P, Mascarenhas J, Bose P, et al. Interferons in the treatment of myeloproliferative neoplasms. Ther Adv Hematol. 2024;15:20406207241229588.Full text  Abstract

112. Barbui T, Vannucchi AM, De Stefano V, et al. Ropeginterferon alfa-2b versus standard therapy for low-risk patients with polycythemia vera. final results of low-PV randomized phase II trial. Blood 2022; 140 (Supplement 1): 1797–9.​

113. 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

114. 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

115. 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

116. 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

117. 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

118. Kiladjian JJ, Chevret S, Dosquet C, et al. Treatment of polycythemia vera with hydroxyurea and pipobroman: final results of a randomized trial initiated in 1980. J Clin Oncol. 2011 Oct 10;29(29):3907-13.Full text  Abstract

119. McMullin MFF, Mead AJ, Ali S, et al. A guideline for the management of specific situations in polycythaemia vera and secondary erythrocytosis: a British Society for Haematology guideline. Br J Haematol. 2019 Jan;184(2):161-75.Full text  Abstract

120. 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

121. Spivak JL, Hasselbalch H. Hydroxycarbamide: a user's guide for chronic myeloproliferative disorders. Expert Rev Anticancer Ther. 2011 Mar;11(3):403-14.Full text  Abstract

122. Björkholm M, Hultcrantz M, Derolf ÅR. Leukemic transformation in myeloproliferative neoplasms: therapy-related or unrelated? Best Pract Res Clin Haematol. 2014 Jun;27(2):141-53. Abstract

123. Gisslinger H, Klade C, Georgiev P, et al. Ropeginterferon alfa-2b versus standard therapy for polycythaemia vera (PROUD-PV and CONTINUATION-PV): a randomised, non-inferiority, phase 3 trial and its extension study. Lancet Haematol. 2020 Mar;7(3):e196-e208. Abstract

124. Gisslinger, H, Klade C, Georgiev P, et al. S196: ropeginterferon alfa-2Bachieves patient-specific treatment goals in polycythemia vera: final results from the proud-PV/continuation-PV studies. HemaSphere. 2022 Jun;6:97-8.

125. 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

126. 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

127. 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

128. 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

129. 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

130. 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

131. 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

132. Kuykendall AT. Treatment of hydroxyurea-resistant/intolerant polycythemia vera: a discussion of best practices. Ann Hematol. 2023 May;102(5):985-93.Full text  Abstract

133. Mazza GL, Mead-Harvey C, Mascarenhas J, et al. Symptom burden and quality of life in patients with high-risk essential thrombocythaemia and polycythaemia vera receiving hydroxyurea or pegylated interferon alfa-2a: a post-hoc analysis of the MPN-RC 111 and 112 trials. Lancet Haematol. 2022 Jan;9(1):e38-48. Abstract

134. Harrison CN, Nangalia J, Boucher R, et al. Ruxolitinib versus best available therapy for polycythemia vera intolerant or resistant to hydroxycarbamide in a randomized trial. J Clin Oncol. 2023 Jul 1;41(19):3534-44.Full text  Abstract

135. 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

136. Passamonti F, Palandri F, Saydam G, et al. Ruxolitinib versus best available therapy in inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): 5-year follow up of a randomised, phase 3b study. Lancet Haematol. 2022 Jul;9(7):e480-92. Abstract

137. 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

138. 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

139. 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

140. 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

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

142. Björkholm M, Derolf AR, Hultcrantz M, et al. Treatment-related risk factors for transformation to acute myeloid leukemia and myelodysplastic syndromes in myeloproliferative neoplasms. J Clin Oncol. 2011 Jun 10;29(17):2410-5.Full text  Abstract

143. Finazzi G, Ruggeri M, Rodeghiero F, et al. Second malignancies in patients with essential thrombocythaemia treated with busulphan and hydroxyurea: long-term follow-up of a randomized clinical trial. Br J Haematol. 2000 Sep;110(3):577-83. Abstract

144. Barbui T, Masciulli A, Marfisi MR, et al. White blood cell counts and thrombosis in polycythemia vera: a subanalysis of the CYTO-PV study. Blood. 2015 Jul 23;126(4):560-1.Full text  Abstract

145. Ahn IE, Natelson E, Rice L. Successful long-term treatment of Philadelphia chromosome-negative myeloproliferative neoplasms with combination of hydroxyurea and anagrelide. Clin Lymphoma Myeloma Leuk. 2013 Sep;13(suppl 2):S300-4. Abstract

146. Alimam S, Bewley S, Chappell LC, et al. Pregnancy outcomes in myeloproliferative neoplasms: UK prospective cohort study. Br J Haematol. 2016 Oct;175(1):31-6.Full text  Abstract

147. Robinson SE, Harrison CN. How we manage Philadelphia-negative myeloproliferative neoplasms in pregnancy. Br J Haematol. 2020 May;189(4):625-34.Full text  Abstract

148. Barbui T. How to manage children and young adults with myeloproliferative neoplasms. Leukemia. 2012 Jul;26(7):1452-7. Abstract

149. 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

150. Sørensen AL, Mikkelsen SU, Knudsen TA, et al. Ruxolitinib and interferon-α2 combination therapy for patients with polycythemia vera or myelofibrosis: a phase II study. Haematologica. 2020 Sep 1;105(9):2262-72.Full text  Abstract

151. Sørensen ALL, Skov V, Kjær L, et al. Combination therapy with ruxolitinib and interferon in newly diagnosed patients with polycythemia vera. Blood. 2022 Nov;140(suppl 1): 6806-7.Full text

152. Guerini V, Barbui V, Spinelli O, et al. The histone deacetylase inhibitor ITF2357 selectively targets cells bearing mutated JAK2(V617F). Leukemia. 2008 Apr;22(4):740-7.Full text  Abstract

153. Rambaldi A, Dellacasa CM, Finazzi G, et al. A pilot study of the histone-deacetylase inhibitor givinostat in patients with JAK2V617F positive chronic myeloproliferative neoplasms. Br J Haematol. 2010 Aug;150(4):446-55.Full text  Abstract

154. Rambaldi A, Iurlo A, Vannucchi AM, et al. Safety and efficacy of the maximum tolerated dose of givinostat in polycythemia vera: a two-part Phase Ib/II study. Leukemia. 2020 Aug;34(8):2234-7.Full text  Abstract

155. Rambaldi A, Iurlo A, Vannucchi AM, et al. Long-term safety and efficacy of givinostat in polycythemia vera: 4-year mean follow up of three phase 1/2 studies and a compassionate use program. Blood Cancer J. 2021 Mar 6;11(3):53.Full text  Abstract

156. ClinicalTrials.gov. A study of bomedemstat (MK-3543) in participants with polycythemia vera (MK-3543-004). NCT05558696. Apr 2024 [internet publication].Full text

157. Ginzburg YZ, Feola M, Zimran E, et al. Dysregulated iron metabolism in polycythemia vera: etiology and consequences. Leukemia. 2018 Oct;32(10):2105-16.Full text  Abstract

158. Kremyanskaya M, Ginzburg Y, Kuykendall AT, et al. PTG-300 eliminates the need for therapeutic phlebotomy in both low and high-risk polycythemia vera patients. Blood. 2020;136(supp 1):33-5.Full text

159. Kremyanskaya M, Kuykendall AT, Pemmaraju N, et al. Rusfertide, a hepcidin mimetic, for control of erythrocytosis in polycythemia vera. N Engl J Med. 2024 Feb 22;390(8):723-35. Abstract

160. ClinicalTrials.gov. A phase 3 study of rusfertide in patients with polycythemia vera (VERIFY). NCT05210790. Mar 2024 [internet publication].Full text

161. ClinicalTrials.gov. A study to evaluate sapablursen (formerly ISIS 702843, IONIS-TMPRSS6-LRx) in patients with polycythemia vera. NCT05143957. Feb 2024 [internet publication].Full text

162. ClinicalTrials.gov. Study to assess SLN124 in patients with polycythemia vera (SLN). NCT05499013. Apr 2024 [internet publication].Full text

163. Serrao A, Breccia M, Napolitano M, et al. A multicenter real-life study on anticoagulant treatment with direct oral anticoagulants in patients with Ph-negative myeloproliferative neoplasms. Am J Hematol. 2020 Dec;95(12):E329-32.Full text  Abstract

164. Barbui T, De Stefano V, Carobbio A, et al. Direct oral anticoagulants for myeloproliferative neoplasms: results from an international study on 442 patients. Leukemia. 2021 Oct;35(10):2989-93.Full text  Abstract

165. ClinicalTrials.gov. AVAJAK: apixaban/rivaroxaban versus aspirin for primary prevention of thrombo-embolic complications in JAK2V617F-positive myeloproliferative neoplasms (AVAJAK). NCT05198960. Jul 2020 [internet publication].Full text

166. Szuber N, Vallapureddy RR, Penna D, et al. Myeloproliferative neoplasms in the young: Mayo Clinic experience with 361 patients age 40 years or younger. Am J Hematol. 2018 Dec;93(12):1474-84.Full text  Abstract

167. Smith CJ, Thomas JW, Ruan G, et al. A population-based study of outcomes in polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the United States from 2001 to 2015: comparison with data from a Mayo Clinic single institutional series. Am J Hematol. 2021 Dec 1;96(12):E464-8.Full text  Abstract

168. Hultcrantz M, Wilkes SR, Kristinsson SY, et al. Risk and cause of death in patients diagnosed with myeloproliferative neoplasms in Sweden between 1973 and 2005: a population-based study. J Clin Oncol. 2015 Jul 10;33(20):2288-95.Full text  Abstract

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

170. Tefferi A, Smock KJ, Divgi AB. Polycythemia vera-associated acquired von Willebrand syndrome despite near-normal platelet count. Am J Hematol. 2010 Jul;85(7):545. Abstract

171. Michiels JJ, Berneman Z, Schroyens W, et al. Platelet-mediated thrombotic complications in patients with ET: reversal by aspirin, platelet reduction, and not by coumadin. Blood Cells Mol Dis. 2006 Mar-Apr;36(2):199-205. Abstract

172. Michiels JJ, Berneman Z, Schroyens W, et al. Platelet-mediated erythromelalgic, cerebral, ocular and coronary microvascular ischemic and thrombotic manifestations in patients with essential thrombocythemia and polycythemia vera: a distinct aspirin-responsive and coumadin-resistant arterial thrombophilia. Platelets. 2006 Dec;17(8):528-44. Abstract

173. Cerquozzi S, Tefferi A. Blast transformation and fibrotic progression in polycythemia vera and essential thrombocythemia: a literature review of incidence and risk factors. Blood Cancer J. 2015;5:e366.Full text  Abstract

174. Tefferi A, Mudireddy M, Mannelli F, et al. Blast phase myeloproliferative neoplasm: Mayo-AGIMM study of 410 patients from two separate cohorts. Leukemia. 2018 May;32(5):1200-10.Full text  Abstract

175. Gupta V, Kim S, Hu ZH, et al. Comparison of outcomes of HCT in blast phase of BCR-ABL1- MPN with de novo AML and with AML following MDS. Blood Adv. 2020 Oct 13;4(19):4748-57.Full text  Abstract

176. Alvarez-Larrán A, Kerguelen A, Hernández-Boluda JC, et al. Frequency and prognostic value of resistance/intolerance to hydroxycarbamide in 890 patients with polycythaemia vera. Br J Haematol. 2016 Mar;172(5):786-93.Full text  Abstract

177. Landtblom AR, Ungerstedt J, Hedlund A, et al. Omalizumab alleviates pruritus in myeloproliferative neoplasms. Haematologica. 2023 Jul 1;108(7):1968-71.Full text  Abstract

Use of this content is subject to our disclaimer