Polycythemia Vera (PV) management often centers around normalizing hematocrit levels. However, each PV patient’s situation is unique, and those without symptoms might not need immediate treatment. Regular doctor check-ups are crucial for all PV patients, regardless of whether symptoms are present. So, Where Is Pv treated and what are the options? Let’s explore the common approaches.
When treatment becomes necessary, typical PV treatments include:
Phlebotomy
Phlebotomy, or blood removal, decreases blood cell count. By reducing blood cells, the blood becomes less viscous and flows more freely. This alleviates symptoms and lowers blood clot risks. Doctors usually perform this procedure to reach specific blood count targets, considering the patient’s gender and other health factors.
Low-Dose Aspirin
Almost all PV patients receive a prescription for low-dose aspirin. Aspirin prevents blood platelets from clumping together, thus minimizing the risk of blood clots that can lead to severe conditions like heart attacks and strokes.
Maintaining a hematocrit level below 0.45 for men and 0.42 for women, alongside low-dose aspirin, is a widely accepted initial treatment, especially for newly diagnosed, low-risk PV patients. This approach is considered non-leukemogenic.
If phlebotomy and low-dose aspirin aren’t sufficient or suitable, or if a patient has a higher blood clot risk, physicians might prescribe medications to lower red blood cell production and ease symptoms. These include:
Hydroxyurea
Hydroxyurea is frequently prescribed for PV patients at high risk of blood clots, particularly based on age or a history of clotting events.
Jakafi (ruxolitinib)
Jakafi is the first FDA-approved medication for PV patients who don’t respond adequately to or cannot tolerate hydroxyurea. Jakafi works by inhibiting JAK 1 and 2 enzymes, which play a role in blood and immune system regulation. It also helps reduce enlarged spleen size (splenomegaly) and the frequency of phlebotomy needs. While most PV patients have the JAK2 mutation, it’s not a requirement to benefit from Jakafi.
IncyteCares offers support for individuals with and without insurance to understand their coverage, explore financial aid, and provide ongoing assistance. Incyte is the developer of Jakafi.
Some PV patients using Jakafi may experience anemia, with or without spleen enlargement. It’s important to have detailed discussions with your doctor when considering treatment choices.
BESREMi (ropeginterferon alfa-2b-njft)
BESREMi gained FDA approval in November 2021 as the first interferon specifically for adults with polycythemia vera, regardless of prior treatments. BESREMi is a long-acting interferon, administered once every two weeks (or every four weeks for stable patients). The PharmaEssentia SOURCE program supports patients prescribed BESREMi. Visit www.pharmaessentiasource.com for details. PharmaEssentia developed BESREMi.
Pegylated Interferon
Pegylated interferon is often used for younger patients needing treatment and women of childbearing age because it has not been linked to birth defects. Pegasys, initially for Hepatitis C, is used “off-label” for MPNs. Clinical trials are ongoing to further assess Pegasys in MPN patients.
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