Polycythemia vera (PV) treatment

Polycythemia vera (PV) is a chronic disease: It's not curable, but it can usually be managed effectively for very long periods. The goal of therapy is to reduce the risk of thrombosis and to ease symptoms by lowering the number of extra blood cells.  

Many treatment options are designed to manage PV by lowering hematocrit levels below 45 percent for men and 42 percent for women. Careful medical supervision and therapy is important to keep the hematocrit concentration at normal levels.  

Treatment planning 

Treatment decisions are based on the patient's risk for clotting complications (thrombosis). 

The two main risk factors for thrombosis are: 

•  A previous clot or clots
• Age 60 years or older 

Every patient’s medical situation is different and should be evaluated individually by a hematologist-oncologist. As you develop a treatment plan with your doctor, it is important to discuss: 

• The results you can expect from treatment
• Potential side effects
• All treatment options, including treatments being studied in clinical trials 

You may find it helpful to bring a loved one with you to your doctor's visits to support you, take notes, and ask follow-up questions. It's a good idea to prepare questions you'd like to ask before you visit your doctor. You can also record your conversations with your doctor and listen more closely when you get home. 

Learn more about communicating with your blood cancer specialist or find a list of suggested questions to ask your healthcare providers.  

Types of treatment for PV 

The following are types of treatment for PV: 

Treatment for low-risk PV 

Patients are generally considered low risk if they are younger than age 60 and have no history of thrombosis.  

Treatment may include: 

• Monitoring for new clots or bleeding
• Manage cardiovascular risk factors 
• Low-dose aspirin: Low-dose aspirin may reduce the risk of blood clots, heart attacks, and strokes. It also helps prevent platelets from sticking together, making it less likely for blood clots to form. Low-dose aspirin consists of 80-100 milligrams of aspirin per day. The most common side effects of aspirin are upset stomach and heartburn.
• Phlebotomy: Most PV patients have their blood drawn regularly to reduce the number of blood cells and decrease blood volume. Phlebotomy is a procedure in which blood is taken from a vein similarly to what is done when donating blood. After phlebotomy, the blood is thinner and less likely to cause “sludging” (which occurs when red blood cells build up along walls of blood vessels). The immediate effect of phlebotomy is to decrease certain symptoms, such as headaches, itchiness, vision problems, ringing in the ears, and dizziness. 

Treatment for high-risk PV  

Patients are generally considered high risk if they are age 60 or older and/or have a history of thrombosis.  

Treatment may include: 

• Monitoring for new clots or bleeding
• Manage cardiovascular risk factors 
• Low-dose aspirin: Low-dose aspirin may reduce the risk of blood clots, heart attacks, and strokes; and helps prevent platelets from sticking together, making it less likely for blood clots to form. The most common side effects of aspirin are upset stomach and heartburn.
• Phlebotomy: Most PV patients have their blood drawn regularly to reduce the number of blood cells and decrease blood volume. Phlebotomy is a procedure in which blood is taken from a vein similarly to what is done when donating blood. After phlebotomy, the blood is thinner and less likely to cause “sludging” (which occurs when red blood cells build up along walls of blood vessels). The immediate effect of phlebotomy is to decrease certain symptoms, such as headaches, itchiness, vision problems, ringing in the ears, and dizziness.
• Cytoreductive therapy (medications to reduce the number of blood cells): High-risk PV patients may be prescribed cytoreductive drugs to reduce the number of blood cells. These drugs may include any or a combination of the drugs listed below:
• Hydroxyurea (Hydrea®)
• Ruxolitinib (Jakafi®)
• Ropeginterferon alfa-2b-njft (Besremi®)
• Peginterferon alfa-2a (Pegasys®) 

For information about the drugs listed on this page, visit our cancer drug listing.  

Clinical trials 

Taking part in a clinical trial may be the best treatment choice for some PV patients. Patient participation in clinical trials is important in the development of new and more effective treatments for PV and may provide patients with additional treatment options.  

There are clinical trials for newly diagnosed patients, patients with advanced disease, and patients who are intolerant of or resistant to their current medications. 

Learn more about clinical trials. 

Get free clinical trial support! Visit our Clinical Trial Support Center (CTSC).

Connect with registered nurses with expertise in blood cancers who can personally assist you or your caregiver through each step of the clinical trial process. 

Managing symptoms 

People with PV experience certain symptoms and risk factors associated with the disease. Here are some ways to manage them:  

Blood clots 

Patients with PV have an increased risk of blood clots compared with the general population. Your doctor may use imaging tests such as ultrasound, CT scans and MRI scans to look for blood clots in your body. If you have a blood clot, your doctor may prescribe an anticoagulant (blood thinner) such as low-molecular-weight heparin.  

Another option is an oral blood thinner, taken by mouth, such as warfarin. For life-threatening blood clots that have already formed, drugs called “thrombolytics” can be given to dissolve them.  

Cardiovascular risk factors  

Patients with PV should focus on their overall health. A heart-healthy lifestyle may decrease the risk of thrombosis. 

Lifestyle changes may include: 

• Controlling your blood pressure
• Keeping your cholesterol under control
• Managing diabetes
• Not smoking
• Working toward a healthy body weight
• Taking your medications
• Exercising  

Discuss healthy lifestyle changes with your doctor. Ask for a referral to a physical therapist for an exercise plan and an oncology registered dietitian for help with nutrition.   

Treatments to reduce itching 

A troublesome symptom that occurs in many PV patients is itchy skin (“pruritus”). Treatment options include: 

• Bathe less frequently
• Bathe or shower in cool water and use a gentle soap
• Avoid hot tubs, heated whirlpools, and hot showers or baths 
• Keep skin well moisturized with lotion and try not to scratch it, because that can damage the skin
• Antihistamines such as diphenhydramine (Benadryl®) or doxepin may help itching that does not go away
• Light therapy (phototherapy) using a medicine called "psoralen" combined with ultraviolet A (UVA) light
• Medications such as gabapentin or pregabalin that block neurotransmitters in the central nervous system from sending signals that trigger itching 

For information about the drugs listed on this page, visit our cancer drug listing.  

Side effects of PV treatment 

The side effects of treatment for PV will depend on many factors, including the type of treatment and dosage, the age of the patient, and coexisting medical conditions. 

Management of side effects is important. Patients should discuss any concerns about side effects with their doctor. Most side effects are temporary and resolve when treatment is completed.  

Learn more about PV treatment side effects and how to manage them.

Special considerations 

Patients with PV have special considerations when it comes to surgery and/or pregnancy. 

Surgery 

Patients with PV have increased risk for bleeding complications after surgery. Because your surgeon may not be aware of your increased risk for bleeding and blood clots, coordination between your surgeon and your hematologist-oncologist is very important. For elective surgeries, it is recommended that your platelet and red blood counts be in normal range before the surgery occurs.  

Aspirin use should be discontinued one week prior to an elective surgical procedure and then restarted 24 hours after the surgery, or when considered acceptable depending on the level of bleeding risk.  

Anticoagulant therapy should likewise be suspended prior to surgery and restarted after surgery, again when considered acceptable depending on bleeding risk. There should also be a plan to minimize the risk for deep vein thrombosis after surgery. 

Pregnancy 

Although MPNs typically develop later in adulthood, some people younger than age 40 are diagnosed with PV. Pregnancies are considered high-risk in younger women with PV.  

If you have PV and are considering a pregnancy, it’s recommended that you meet with an obstetrician specializing in high-risk pregnancies before you become pregnant. 

Your doctor may recommend taking aspirin during and shortly after your pregnancy.  

Sometimes, use of an injectable anticoagulant called enoxaparin (Lovenox®) is recommended for six weeks following the delivery of the child. 

Hydroxyurea should not be taken during pregnancy or while breastfeeding, as it may harm your baby. If you are taking hydroxyurea, you may switch to interferon during your pregnancy. 

Treatment outcomes 

For some people with PV, the disease remains stable for many years. Many people’s life expectancy is the same as it would be if they did not have PV. With careful medical supervision and therapy, PV can usually be managed effectively for a long time. In some cases, however, it may progress to another type of blood disease, such as myelofibrosis (MF) or acute myeloid leukemia (AML). 

It is important to know that outcome data can show how groups of people with an MPN responded to treatment in the past, but it cannot always determine how any particular person will respond. For these reasons, patients are advised to discuss information about survival with their doctors.  

Follow-up care 

Find more information about follow-up care, including what to expect, long-term and late effects of treatment, survivorship clinics, and other resources, such as The National Comprehensive Cancer Network (NCCN) treatment guidelines.  

Use the Survivorship Workbook to collect all the important information you need throughout diagnosis, treatment, follow-up care, and long-term management of a blood cancer.