There is currently no cure for polycythemia vera (PV), but researchers are looking at new treatments that may help better manage and control the progression of the condition.
Polycythemia vera (PV) is a rare, chronic blood disease that can cause thrombosis (blood clots), itchy skin, and several other symptoms.
Although there is currently no cure, improvements in treatments over the past few years have helped raise the life expectancy of people with PV to a median of 20 years.
Read on for more information about some of the newer treatments being studied and implemented.
According to the
At first, PV may not cause symptoms. In many cases, a routine blood test finds evidence of PV before any symptoms present.
Treatment options typically focus on a few different areas of the disease. According to the Genetic and Rare Diseases Information Center (GARD), the primary goal of treatment is to help prevent thrombosis (blood clots) from occurring. Blood clots can lead to conditions such as heart attack or stroke if they travel to the heart or brain.
The secondary goal of treatment is to help improve quality of life. Doctors may prescribe medications or make recommendations to help prevent itchiness or depression symptoms.
Current research is looking more closely into the use of JAK2 inhibitors to treat PV. Treatments focusing on the JAK2 gene may help with improving symptoms of the disease.
According to one
Both the above study and the Leukemia & Lymphoma Society state that treatments are split into one of two categories: low risk patients or high risk patients. Low risk patients are people younger than 60 years of age with no history of blood clots, while high risk patients are people who are 60 years or over or who have a history of blood clots.
The Leukemia & Lymphoma Society add that therapies are also used to:
- reduce hematocrit concentration, or the percentage of red blood cells in the blood, to normal or close-to-normal levels
- decrease other PV-related symptoms
- lower platelet counts, if their concentrations are high
Standard treatments
Treatment options for both high and low risk patients include phlebotomy (regular drawing of blood to reduce the amount of blood cells in the body) and low dose aspirin (which can help reduce the risk of blood clots).
Specific medications
The Leukemia & Lymphoma Society also state that doctors will likely prescribe one or more medications to help reduce the number of blood cells in the bodies of high risk patients.
Some common medications used for this include:
- busulfan (Myleran)
- chlorambucil (Leukeran)
- hydroxyurea (Hydrea)
- interferon alfa (Intron A)
- ruxolitinib (Jakafi)
Additional therapies
According to the GARD, doctors may also work with people to help reduce symptoms of itching. They state that no single treatment works for every person.
The Leukemia & Lymphoma Society explain that common treatments for itchiness include:
- applying topical or oral antihistamines
- taking less frequent baths or showers
- using cool water in the shower or bath
- using gentle soaps or other skin products
- avoiding heated whirlpools, hot showers or baths, and hot tubs
- trying light therapy
- not scratching the skin
- using moisturizers
Researchers are exploring new treatment options. According to the
- Selective serotonin reuptake inhibitors: These are typically used for depression, but they may also help reduce itchiness.
- JAK2 inhibitors: These help block a mutated JAK2 gene from producing too many blood cells.
- Imatinib mesylate: This is typically used for leukemia, but it may also help decrease spleen size and reduce the number of phlebotomies a person needs.
The Leukemia & Lymphoma Society also point to additional clinical studies, including those investigating:
- Givinostat: This is a histone deacetylase medication that helps prevent abnormal cell growth. It may be a second line of treatment for people whose bodies do not tolerate first-line treatments well.
- Peginterferon alfa-2a (Pegasys): This is a JAK2 inhibitor targeting the JAK2 gene.
- Idasanutlin: This is an MDM2 inhibitor that blocks certain protein interactions.
Another recent study, called the PTG-300 Polycythemia Vera Study, is looking at the medication PTG-300. The medication is designed to help maintain iron balance in the bodies of people with PV. Its goal is to improve quality of life and control the number of red blood cells in the body.
Other studies are either recruiting or wrapping up. Anyone interested in participating in a clinical trial can visit ClinicalTrials.gov for a list of PV-related studies.
A person should talk with their doctor about participating in a clinical trial. They may be able to advise the person on where to find a study near them and help determine whether they would be a good candidate.
There is currently no cure for PV, but the disease is not necessarily a death sentence.
According to the Leukemia & Lymphoma Society, the median life expectancy after diagnosis is 20 years. Although other health conditions, age, and overall health status can negatively affect a person’s survival rate, some with the condition may live close to a normal life expectancy.
Ongoing medical treatment is important to improving both symptoms and life expectancy. A person should not stop taking medications designed to help reduce blood clots without talking about it with their doctor first.
PV treatments currently focus on helping prevent thrombosis (blood clots) and negative complications from them, such as heart attack and stroke.
Future treatments using JAK2 inhibitors may help with improving quality of life and increasing life expectancy even further.
A person who is interested in taking part in a clinical study should talk with a doctor to find out about current studies taking place.