What is Polycythemia vera (PV)?

Polycythemia vera (PV) is a type of blood cancer. PV is primarily a result of a mutation in blood-forming stem cells, which causes blood cells (particularly red blood cells) to produce uncontrollably. PV is one of three myeloproliferative neoplasms (MPNs). 

Signs and Symptoms of PV

The increase in red blood cells can cause the blood to thicken and clot, which causes signs and symptoms of PV to occur. In early stages, patients have few symptoms; however as Polycythemia vera progresses patients may experience the following:

  • Blood clot 
  • Headache
  • Excessive sweating
  • Blurred vision or blind spots
  • Dizziness
  • Itchiness, especially after a warm shower or bath
  • Redness or a purplish appearance of skin
  • Peptic ulcers
  • Bloat or a feeling of fullness due to enlarged spleen
  • Congestive heart failure or angina
  • Gout/inflammation of joints

Diagnosing PV

Blood tests are used to diagnose Polycythemia vera. If you have PV, bloods tests may indicate: 

  • An increase in red blood cells, platelets and white bloods cells
  • An elevated hematocrit (the proportion of red blood cells in the blood) 
  • An increase in hemoglobin concentration (a protein found in red blood cells)
  • Low levels of erythropoietin (a hormone that controls red blood cell production)

Additional tests may include:

  • A bone marrow biopsy and aspirate
  • Testing for the presence of JAK2 mutation or other genetic mutations 

Treatment of PV 

There may be several health care providers involved in your care including a hematologist/oncologist, nurse, nurse navigator, oncology social worker and pharmacist. Each of these professionals can help guide you to information and resources that may help you both during and after your treatment. If you do not meet these professionals upfront, ask your doctor how you might arrange an appointment with them. In addition, you may want to contact your insurance carrier to ask for a case manager to be assigned to you to assist in managing your treatment coverage options. 

There are several approaches to treatment depending on your PV diagnosis and its progression.   

There are various drugs used in the treatment of PV, and many of them have very different approaches to slowing the disease process and/or controlling the effects of the disease. With any drugs, side effects will likely exist; your doctor or nurse should review these with you before starting.

Options may include: 

  • Watch & Wait, is when patients who are symptom and sign free and exhibit slow disease progression are not treated at the time of diagnosis. Some people remain stable and symptom free for many years. However, it is very important that individuals be closely monitored through regular medical checkups and examinations to detect signs or symptoms of disease progression. 
  • Phlebotomy, which removes blood from a vein in order to reduce the amount of overall red blood cells in the body. Many people diagnosed with PV may begin with this type of treatment.
  • JAK Inhibitors, there are now FDA (Food & Drug Administration) approved therapies for the treatment of PV. 
  • Chemotherapeutic Agents, which may reduce the platelet count, and are usually given by mouth (orally). Some agents may be given intravenously (into the vein). 
  • Aspirin, taken orally, in low doses. Can help reduce the risk of clotting. 
  • Biological therapies, which may also help reduce platelet counts, and are usually given by injection. 
  • Clinical Trials, because doctors are still trying to find more effective treatments for PV, clinical trials are an important option to consider. Clinical trials are research studies to test promising new drugs or treatment approaches. Participating in a clinical trial may give you the opportunity to be among the first to benefit from the most advanced treatment available. If you are interested in a clinical trial, ask your healthcare team about studies that you might qualify for. More information on clinical trials and how to participate in one. 

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