Polycythemia Vera Clinical Considerations

Traditional Considerations When Assessing Risk

Recommendations for the management of polycythemia vera are aimed at lowering the risk of thrombosis.1

Assessment also includes evaluation of general cardiovascular risk factors.

Clinical Need in Polycythemia Vera

Goals that drive clinical decision-making for PV include1,3,4:

  • Maintaining an Hct level of <45%
  • Managing the complications of thrombosis and hemorrhage
  • Controlling leukocyte and platelet counts
  • Managing disease-related symptoms and splenomegaly
  • Lowering the risk of thrombotic events without increasing the risk of leukemic transformation
  • Controlling progressive bone marrow hyperplasia

Phlebotomy is usually the starting point of treatment in patients with PV, in addition to therapy with low-dose aspirin.1,5 Low-dose aspirin has been shown to prevent thrombotic complications in patients with PV.6

Cytoreductive therapy is utilized in patients with high-risk PV. Cytoreductive therapy may also be helpful in patients who have difficulty with phlebotomy, who have symptomatic or progressive splenomegaly, or who experience severe disease-related symptoms.1

Risk stratification in polycythemia vera1,2
High-risk (presence of one of the following):
  • Advanced age (>60 years)
  • History of thrombosis
Other comorbid risk factors:
  • Cardiovascular risk factors, including:
    • Arterial hypertension
    • Hypercholesterolemia
    • Diabetes mellitus
    • Smoking
References

1. Barbui T, Barosi G, Birgegard G, et al. J Clin Oncol. 2011;29:761-770. 2. Falanga A, Marchetti M. Hematology Am Soc Hematol Educ Program. 2012;2012:571-581. 3. Alvarez-Larrán A, Pereira A, Cervantes F, et al. Blood. 2012;119:1363-1369. 4. Barosi G, Mesa R, Finazzi G, et al. Blood. 2013;121:4778-4781. 5. Marchioli R, Finazzi G, Specchia G, et al; for the CYTO-PV Collaborative Group. N Engl J Med. 2013;368:22-33. 6. Landolfi R, Marchioli R, Kutti J, et al; for the European Collaboration on Low-Dose Aspirin in Polycythemia Vera Investigators. N Engl J Med. 2004;350:114-124.

 
Resources
The potential impact of a target hematocrit level of <45% on major thrombosis or cardiac death.
By: MPN Connect
An animated video highlighting PV pathophysiology and mechanisms of disease.