Managing polycythemia vera
What are the goals of PV treatment?
Treatment goals
One of the main goals of treating PV is to keep your hematocrit (HCT, the percentage of red blood cells in your blood) below 45%. Doing so helps lower your risk of blood clots, heart attack, and other serious problems.
In a large clinical study called CYTO-PV, people with polycythemia vera (PV) who had HCT levels above the 45% threshold, specifically 45%-50%, were 4 times more likely to have a blood clot or die from a heart-related problem compared to those who kept it below 45%.
Other important treatment goals include reduction of PV symptoms and increasing quality of life so that you can still pursue your daily activities.
The Doctor Discussion Guide can help guide conversations with your doctor
Hear from Julie-Ann, a real person living with PV
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0:06: Music has been a part of my life since I was about 4 years old, and the piano is where I pour my soul.
0:12: It's the one thing that I feel like is constant for me.
0:17: I found out that I had PV in March of 2024.
0:24: I didn't know what polycythemia vera was.
0:27: I didn't know what therapeutic phlebotomy was.
0:32: I had started the treatments with one doctor, and then when I went to my new doctor, she didn't see any evidence of PV.
0:42: She also was one of those people who didn't really think that it was anything to worry about.
0:48: So I stopped doing the phlebotomy for several months.
0:51: And I remember calling my doctor and saying, you know, can we just do another blood test?
0:57: When the test came back, it was very evident that I had PV.
1:04: I tend to lose focus kind of easy these days, and I don't really like that.
1:12: I don't like that I feel dizzy a lot.
1:15: I don't like that I have headaches.
1:17: I don't like that I'm tired.
1:19: I lost the ability to do things that had been like breathing for me.
1:25: I have a song that I played on the piano.
1:26: It's my very, very favorite thing to play.
1:29: And it was like I couldn't make the connection between my brain and my fingers.
1:36: I couldn't play it, and it really scared me, and I don't like that I worry about what if it doesn't get better.
1:45: And it's been a long road.
1:48: I've seen situations where people have this and they've gone untreated for way too long. But positively,
1:55: it's given me a freedom to speak out about my health, to make sure that other people understand what PV is.
2:09: I really want anyone who has this to know that it is serious and that there is good information out there about it.
2:18: If someone's doctor is not giving them the information.
2:23: I really want to encourage any patients, and I guess this would go for anybody,
2:29: ask questions,
2:30: find out everything you can
2:33: about whatever it is that your doctor is telling you that you have.
Current treatment options for PV
A primary goal of treatment is to consistently maintain your percent of red blood cells (HCT levels) below the 45% threshold.
Therapeutic phlebotomy and low-dose aspirin
Therapeutic phlebotomy and low-dose aspirin
Initial treatment may include low-dose aspirin to help lower the risk of blood clots, typically along with therapeutic phlebotomy.
Therapeutic phlebotomy, sometimes just called phlebotomy, is usually the first treatment you will have for your PV.
While a standard phlebotomy is used for diagnostic purposes and blood donation, therapeutic phlebotomy is specifically used to treat your PV as an interim treatment for temporary hematocrit reduction. This helps lower the number of red blood cells and reduce the thickness of your blood.
You may need therapeutic phlebotomies regularly, especially at the beginning of treatment. Over time, the schedule may change depending on your blood counts. Therapeutic phlebotomies are a common part of PV care, but when they’re unpredictable, they can add stress and uncertainty to daily life.
People with PV who need phlebotomies for therapeutic purposes, as opposed to standard phlebotomies, may need to have treatments as many as 8 times a year, with each appointment taking several hours. Travel, scheduling, and recovery time can interfere with work, family, and routines. Many people also report feeling tired or dizzy afterward, and repeated blood draws can make veins harder to access.
If symptoms worsen or phlebotomy alone isn’t enough to control PV, your doctor may suggest adding other treatments, such as cytoreductive therapies.
Cytoreductive therapies (eg, hydroxyurea, interferons, JAK inhibitors)
Cytoreductive therapies (eg, hydroxyurea, interferons, JAK inhibitors)
Cytoreductive therapies are medications that help slow down how many blood cells your body makes.
Hydroxyurea, or HU, is an oral chemotherapy that is frequently used in PV, although it can be difficult to tolerate.
Even when on hydroxyurea, many people still need therapeutic phlebotomy at times to keep HCT under control.
JAK inhibitors and interferon therapy are other treatment options for advanced PV, and are mainly used after trying other therapies.
Cytoreductive therapies may be initially recommended if you’re over 60, have had a blood clot, or if phlebotomy alone isn’t keeping your PV under control.
Some cytoreductive therapies require a period of time before they begin to work, which could be upwards of ~8 months, so you need to stay vigilant with monitoring during this time.
Many currently approved cytoreductive therapies are associated with high rates of discontinuation due to side effects, which occur in up to 1/3 of those with PV.
Nearly 4 out of 5 people with PV did not consistently maintain their HCT below the 45% threshold, despite their current treatment.