How Does Polycythemia Vera Progress?
Polycythemia vera, also called “PV,” is a chronic blood disorder in which a person’s bone marrow (the spongy tissue located in the center of your bones) makes too many red blood cells. People with PV also make too many white blood cells (the cells that fight infection) and platelets (the cells that help stop bleeding by forming clots).
A mutation of the JAK2 gene causes PV. The JAK2 gene creates a protein that helps your bone marrow make blood cells, and a change in the gene (called a mutation) leads to increased blood cell counts. Experts are not sure why some people develop this mutation.
While PV is not inherited (meaning the mutated gene is not passed down from a parent to his or her child), research suggests that some people have a genetic vulnerability to developing PV.
In other words, for some unknown reason, certain people are more likely than others to acquire the JAK2 mutation. Take a look at this animation to learn how the mutation affects bloodflow and red blood cell count.
Red blood cells carry oxygen to the rest of your body via the bloodstream. When there are too many of them, your blood becomes thicker than normal, as shown.
Since thicker blood flows more slowly, a person with PV is at risk of not getting sufficient oxygen to their organs, causing various symptoms. Luckily, the right treatment can manage these as well as PV progression.
Although PV isn’t curable, there are multiple treatment options available for stalling its progression and managing symptoms for a long time. Understanding why your doctor recommends a specific one is an empowering way to play an active role in your care.
If you're not sure where to start the conversation, this doctor discussion guide can help. Download it for a refresher on PV-related vocabulary and use the commonly asked questions to learn as much as you can about living with polycythemia vera.
Your Therapy Options
There are several categories of therapies available. In addition to taking them as prescribed, healthy diet, exercise, seeing your primary care doctor for preventive health care, and managing cardiovascular factors like diabetes or obesity are also essential.
Each therapy is prescribed under a unique situation, so not all options are right for everyone. Take this quiz to learn more about the circumstances each may be prescribed under.
Oral hydroxyurea is prescribed when you cannot tolerate or do not respond well to other drugs that reduce blood cell production.
The Benefit of Getting Treated
Learning you have been diagnosed with PV can be distressing and naturally, you may wonder, "Will I be OK?" Although there is no cure, it’s still important to explore and get treatment. With the right one, you can successfully stall PV’s progression in your body and manage symptoms for a long time.
With treatment, survival increases from 6 to 18 months to 13 years or more!
Treatment also helps you manage symptoms and reduces your risk of developing complications and comorbidities, so you can maintain a higher quality of life. Stroke, heart attack, blood clots, or pulmonary embolus (blood clot in the lung) are serious PV complications and can occur because the blood has difficulty moving through the vessels. With treatment, your risk of developing them is reduced.
Managing Signs and Symptoms
PV symptoms result primarily from an elevated red blood cell count. That said, a high number of platelets and white blood cells also play a role in certain symptoms and complications.
It’s important to note that the course of PV is quite variable, meaning some people have a mild disease with few symptoms whereas others have a more severe, debilitating disease. Those with few symptoms of PV are often only diagnosed incidentally after a routine medical visit.
And while nonspecific symptoms like headache, dizziness, facial flushing, weakness, excessive sweating, and unusual tiredness are common, there are also specific symptoms to note, along with helpful strategies for managing them.
Vision ChangesVision problems ranging from blurry or double vision to transient blindness may occur in PV. They are a result of delayed blood flow to the retina, since the blood of people with PV is thick and moves sluggishly and slowly.
If you or a loved one has PV and are experiencing vision changes, especially if you have transient blindness, be sure to contact your oncologist right away. Early treatment with therapies that reduce the number of red blood cells is important to prevent possible permanent eye damage.
Teferri A. Prognosis and Treatment of Polycythemia Vera. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.
Parnes A, Ravi A. Polycythemia and thrombocytosis. Prim Care. 2016;43(4):589-605. doi:10.1016/j.pop.2016.07.011
Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management. Am J Hematol. 2019;94(1):133-143. doi:10.1002/ajh.25303