Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by overproduction of red blood cells by the bone marrow, which may be accompanied by elevated white blood cells and platelets. This can result in increased blood viscosity and elevated risk of thromboembolic events [1].

PV is a chronic condition requiring ongoing medical management. Your healthcare provider can help develop an individualized treatment plan based on your specific clinical situation [2].

PV is most commonly associated with a JAK2 gene mutation, which contributes to dysregulated blood cell production. Current treatment approaches focus on controlling disease manifestations rather than addressing the underlying genetic mutation [3].

Patients should discuss with their healthcare provider what this means for their individual long-term management strategy [4].

Treatment goals in PV typically include [1,5]:

• Maintaining hematocrit levels within target range (generally <45%)

• Reducing risk of thrombotic complications

• Managing disease-related symptoms

• Monitoring for potential disease progression

Treatment Approaches May Include:

Your healthcare provider may recommend one or more of the following approaches based on your individual risk profile:

Phlebotomy: Periodic blood removal to maintain target hematocrit levels

Aspirin therapy: Low-dose aspirin may be prescribed to help reduce thrombotic risk in appropriate patients

Cytoreductive medications: Certain medications may be prescribed to help manage blood cell production

Risk factor management: Addressing cardiovascular risk factors, maintaining hydration, and smoking cessation

Note: All treatment decisions should be made in consultation with your healthcare provider based on your individual clinical situation, risk factors, and treatment goals.

PV requires regular monitoring, which may include:

• Periodic blood counts and hematocrit measurements

• Assessment of symptoms and overall health status

• Monitoring for potential complications

• Periodic evaluation of treatment effectiveness

Your healthcare provider will determine the appropriate monitoring schedule for your situation [1].

Some patients may achieve what is referred to as remission, where blood counts normalize and symptoms improve with treatment. However, PV remains a chronic condition requiring ongoing monitoring even during periods of disease control [1].

Speak with your healthcare provider about realistic treatment goals and outcomes for your individual case.

Allogeneic stem cell transplantation is a treatment modality that may be considered in select cases. This procedure is associated with significant risks including but not limited to: infection, graft-versus-host disease, organ toxicity, and treatment-related mortality. Due to the risk profile, this approach is generally reserved for specific clinical situations as determined by your healthcare team [6].

Patients considering any treatment option should have a thorough discussion with their healthcare provider about potential risks and benefits.

Research continues to investigate various therapeutic approaches in PV, including:

• Agents targeting the JAK-STAT signaling pathway

• Investigations into mechanisms of inflammation and fibrosis

• Studies evaluating long-term disease management strategies

These research efforts are ongoing and their clinical applications continue to be evaluated [7].

Note: Investigational treatments are not approved for use and their safety and efficacy have not been established. Discuss current approved treatment options with your healthcare provider.

Many patients with PV work with their healthcare team to manage their condition over the long term. Important considerations include:

• Adherence to prescribed treatment plans

• Regular medical follow-up and monitoring

• Lifestyle measures as recommended by your healthcare provider

• Open communication with your healthcare team about symptoms or concerns

• What are my individual treatment goals?

• What treatment approach is recommended for my situation?

• What are the potential risks and benefits of recommended treatments?

• How often will I need monitoring and follow-up?

• What symptoms should prompt me to contact you?

• Are there clinical trials I should consider?

• What lifestyle modifications may be helpful in my case?

1. Vannucchi AM. How I treat polycythemia vera. Blood. 2014;124(22):3212-3220.

2. Fox S, Griffin L, Harris DR. Polycythemia vera: rapid evidence review. Am Fam Physician. 2021;103(11):680-687.

3. Griesshammer M, Gisslinger H, Mesa R. Current and future treatment options for polycythemia vera. Ann Hematol. 2015;94(6):901-910.

4. Vannucchi AM, Guglielmelli P. Current treatment approaches for PV and ET. Hematology Am Soc Hematol Educ Program. 2017;2017(1):480-488.

5. Lengfelder E, Merx K, Hehlmann R. Diagnosis and therapy of polycythemia vera. Semin Thromb Hemost. 2006;32(3):267-275.

6. Bewersdorf JP, et al. Moving toward disease modification in polycythemia vera. Blood. 2023;142(22):1859-1870.

7. Damahe A, Sahu KK, Sahu A. Novel approaches for cure and treatment in polycythemia. International Journal of Scientific Research and Technology. 2025.