Treatment

Treatment of Polycythemia vera will depend on certain things like overall health and age, severity of disease and likelihood of progression, and other risk factors. 

Those of younger age, typically under 60 with no history of clotting disorders or events are generally considered lower risk than those of more advanced age and/or those with previous clotting events. 

Unfortunately there is no definitive cure for PV. There are a number of treatments available to lessen symptoms and improve outcomes. PV carries a fairly optimistic prognosis unlike some cancers and hematological malignancies. On average, most people can live twenty years past their diagnosis date if they remain committed to their health and treatment plan. The most common cause of death in PV patients is clotting, so it is essential to be on top of things post-diagnosis. Other complications include progressing disease which may transform into myelofibrosis, AML (Acute Myelogenous Leukemia), or MDS (Myelodysplastic Syndrome). The treatments available generally do not lessen the risk of progression towards AML or MDS. Roughly 10 percent of patient's with PV may experience transformation info AML or MDS. Patients who progress to AML or MDS will see shorted expectations and worse prognosis. 

Therapeutic Phlebotomy -- It is exactly as it sounds. When you go to the doctor and have labs drawn, a nurse or phlebotomist will perform a phlebotomy or "venipuncture" to acquire blood from the patient for testing. This is essentially the same idea. Therapeutic phlebotomy involves a venipuncture in which blood is removed from the patient in order to relieve many of the symptoms and potential future problems of having hyperviscous blood. Up to 500mL is removed during a single sitting (the same amount removed during blood donation). Lesser amounts may be taken depending on the patient's cardiovascular heath and age. Initially, phlebotomies will take place as often as possible (once a day, every other day) until the patient's hematocrit reaches a normal 45%. From there a plan will be devised and often patients will only need phlebotomies once every month or every other month. It is possible however that due to frequent phlebotomies, iron within the blood may decrease. This treatment alone can buy the patient a large amount of their life back. Many of the symptoms listed here will be alleviated by having normal blood levels. This does not prevent transformation into other cancers such as AML/MDS however. For low risk patients, this is one of the only treatments that may be necessary. No drugs or chemotherapies needed.

Aspirin therapy -- for low risk patients without a history of bleeding issues, low dose aspirin may be prescribed. Aspirin inhibits platelet function which allows for better management of clotting in PV patients who have a much higher risk of clotting. Platelets are fundamental in the clotting process, thus why this is beneficial. 

Antihistamines -- This can be beneficial in treating patients seeing an increase in itchiness, especially after warm water contact

Hydroxyurea -- Hydroxyurea (HU) or hydroxycarbamide is a drug taken by mouth that is generally prescribed to patients with higher risk factors. It is a myelosuppressive drug which means that it suppresses the bone marrow from producing cells. It does this by inhibiting the production of DNA within the cells, which in turns means the cells can no longer replicate and grow. This will eventually produce lower RBC counts as well as other cells as well. One would expect platelet counts to drop as well (since elevated platelet counts are often seen in PV). One caveat to HU is that there may be a link to hydroxyurea use and progression to leukemia. Most studies show this risk is very small or non-existent. Often times, symptomatic Sickle Cell Anemia patients will be on this drug as well. 

Ruxolitinib -- also known as Jakafi or Jakavi is a janus kinase inhibitor. It will selectively inhibit JAK1 and JAK2 enzymes from functioning which will assist in inhibiting those with JAK2 enzymes that have formed from mutated JAK2 genes. If you remember from earlier reading on the main page, this JAK2 mutation causes it to be stuck "on" and signals to the bone marrow to perpetually create cells. Ruxolitinib would help shut down this activity and potentially help the bone marrow cease the overproduction of cells. Generally this drug is given for patients who do not respond to Hydroxyurea or cannot receive Hydroxyurea due to allergy or other negative response. 

Ropeginterferon Alfa-2b-njft (BESREMi) -- A form of Interferon alfa. It interacts with receptors within the bone marrow to modulate cell signaling the promotes "anti-proliferative" effects as well as immune modulating and pro-apoptotic (cell death) events. This was recently approved by the FDA in 2021 as an official treatment.