Written by Neal Dave, PharmD Texas Oncology
Treatment of Polycythemia Vera can be challenging, and identifying patients that are having a proper response to treatment is essential to preventing cardiovascular events. Establishing follow ups and lab reviews are recommended to ensure positive outcomes.
Background PCV is a primary polycythemia in which the bone marrow produces too many red
blood cells, white blood cells, and platelets. An acquired mutation in the janus activating kinase 2
(JAK2), found in most PCV patients, allows these cells to proliferate leading to the abnormally
high red blood cell count. Common treatments for PCV are aspirin, phlebotomy, hydroxyurea,
interferon (not commonly used), or a JAK2 inhibitor, ruxolitinib. Hydroxyurea (HU) is considered
the gold standard treatment to start with for high risk patients (see below). Patients that are
taking hydroxyurea and still have high blood counts or cannot tolerate it may benefit with
treatment with ruxolitinib.
|Low Risk PCV patient characteristics:||Age < 60 and no previous history of blood clots|
|High Risk PCV patient characteristics:||Age > 60 or previous history of a blood clot|
One of the treatment goals for PCV is to reduce the cardiovascular risk of patients. Typically, clinicians try to maintain a HCT < 45% and often a target < 42% for women1. A study published in the NEJM showed that patients with a HCT in the 45-50% range had a 4 times greater risk of cardiovascular events than patients with a HCT less than 45%2.
Proper follow up and review of CBCs are required when patients are started on Hydroxyurea. Dose adjustments and possibly additional phlebotomies should be considered for these patients. Identifying the proper time to switch to another therapy is important to help manage the disease Anywhere between 20-60% of patients remain on hydroxyurea even though they are not having a proper response3.
- Review CBC with refill every to ensure HCT is < 45%
- Assess patients for adverse events and document in EMR
- Use the MPN-SAF total symptom score scale (4)
- If HCT > 45 or symptoms are worse:
- Low risk patients not taking HU:
- Consider recommending HU for low risk patients (1-2g/ day in 1-3 divided doses)
- High Risk patients
§ If patient has been on HU for longer than 12 weeks and is still requires phlebotomies then recommend possibly switching to second line therapy.
- Ask prescriber if they want to consider ruxolitinib 10mg BID.
Document intervention in EMR
Patient Centered Activities
Stress importance of adherence
Schedule follow up calls
Maintain adherence to treatments of secondary health conditions
(high blood pressure, diabetes, high cholesterol, history of blood clots)
- Laboratory monitoring will be required with refills
- Possibility of dose adjustments
- Importance of staying hydrated
- Infection prevention- Call clinic for any fever > 100.4F
- Monitoring Skin for patients on ruxolitinib:
- Important to notice all skin lesions
- Examine skin at baseline
- Make note of any new lesions that arise while on therapy
- Marchioli et al, N Engl J Med 2013; 368:22-33, January 3, 2013
- Griesshammer M, Gisslinger H, Mesa R. Current and future treatment options for polycythemia vera.
- Ann Hematol. 2015;94(6):901-910.
- Scherber et al Blood, 14 July 2011 v118, n2
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