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PV is a trilineage disease

PV is a myeloproliferative disorder characterised by trilineage haematopoietic cell growth1,2

  • PV is characterised by excessive erythrocytosis and elevated white blood cell (WBC) and platelet counts2

Uncontrolled trilineage proliferation drives thrombotic complications1-4

Excessive myeloproliferation results in multiple physiologic effects associated with increased risk and burden of disease5

The risks and burden of PV5

The PV diagnostic criteria highlight the trilineage characteristics of PV6

Overactive JAK pathway signalling drives the pathogenesis of PV8-10

Pathogenesis of PV

  • Overactive JAK signalling results in trilineage proliferation and can increase production of inflammatory cytokines8-10
  • Almost all patients (>99%) with PV harbor a mutation in the JAK2 gene11

Next: Inadequate Control in PV

AML=acute myeloid leukaemia; BM=bone marrow; HCT=haematocrit; MF=myelofibrosis; RBC=red blood cell.

References:

  1. Barbui T, Finazzi G, Falanga A. Myeloproliferative neoplasms and thrombosis. Blood. 2013;122(13):2176-2184.
  2. Falanga A, Marchetti M. Thrombotic disease in the myeloproliferative neoplasms. Hematology Am Soc Hematol Educ Program. 2012;2012:571-581.
  3. Stuart BJ, Viera AJ. Polycythemia vera. Am Fam Physician. 2004;69(9):2139-2144.
  4. Michiels JJ, Berneman Z, Van Bockstaele D, van der Planken M, De Raeve H, Schroyens W. 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. 2006;32(3):174-207.
  5. Stein BL, Moliterno AR, Tiu RV. Polycythemia vera disease burden: contributing factors, impact on quality of life, and emerging treatment options. Ann Hematol. 2014;93(12):1965-1976.
  6. Tefferi A, Vardiman JW. Classification and diagnosis of myeloproliferative neoplasms: The 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia. 2008;22:14-22.
  7. Thiele J, Kvasnicka HM. The 2008 WHO diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis. Curr Hematol Malig Rep. 2009;4(1):33-40.
  8. Levine RL, Pardanani A, Tefferi A, Gilliland DG. Role of JAK2 in the pathogenesis and therapy of myeloproliferative disorders. Nat Rev Cancer. 2007;7:673-683.
  9. Quintás-Cardama A, Kantarjian H, Cortes J, Verstovsek S. Janus kinase inhibitors for the treatment of myeloproliferative neoplasias and beyond. Nat Rev Drug Discov. 2011;10(2):127-140.
  10. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon, France: IARC Press; 2008.
  11. Tefferi A. Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1. Leukemia. 2010; 25:1059-1063.