Polycythemia Vera: Causes, Symptoms, and Treatment

Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 23, 2026.

What kind of doctor treats Polycythemia Vera?

A hematologist, a doctor who treats blood diseases, usually leads care for polycythemia vera. An oncologist, a doctor who treats cancers, may also help, since the condition starts in the bone marrow. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.

What is polycythemia vera?

Polycythemia vera (PV) is a bone marrow disease that leads to an abnormal increase in the number of blood cells. The bone marrow is where the body makes blood cells. Red blood cells are the most affected type. White blood cells and platelets, the cells that help blood clot, may also be higher than normal.

The extra cells make the blood thicker than normal. Thick blood cannot flow through small blood vessels the way it should. PV is rare and occurs more often in men than in women. It usually develops slowly and is not usually seen in people under age 40.

Symptoms

In many people, PV does not cause symptoms in the early stages. Thicker blood that cannot flow normally can lead to symptoms such as:

  • Headache, dizziness, and feeling tired all the time.
  • Itchy skin, especially after a warm bath.
  • Red skin color, especially on the face.
  • Vision problems, ringing in the ears (tinnitus), and joint pain.
  • A full feeling in the left upper belly from an enlarged spleen.
  • Shortness of breath, or trouble breathing when lying down.
  • Signs of blood clots in veins near the skin surface (phlebitis).

Thick blood also makes abnormal blood clots more likely. A clot can block blood flow in an artery or a vein. Other problems PV can cause include stomach or gut bleeding, gout (painful swelling of a joint), heart failure, and myelofibrosis, in which scar tissue replaces the bone marrow. A type of blood cancer called acute myelogenous leukemia is also possible.

Causes and who gets it

PV is often linked to a gene change called JAK2 V617F. The JAK2 gene gives cells instructions for growing and dividing. Nearly all people with PV have a change in this gene. The reason for the gene change is unknown.

Most cases of PV are not inherited. The gene changes are acquired during a person's lifetime and are present only in certain cells. In rare cases, PV runs in families. PV typically develops in adulthood, around age 60, and men are affected more often than women.

How doctors diagnose it

Your provider will do a physical exam. Tests may include a complete blood count, a bone marrow biopsy, and a genetic test for the JAK2 V617F change. A bone marrow biopsy takes a small sample of marrow to study. Doctors may also check the oxygen level in your blood and your vitamin B12 level. They must tell PV apart from other causes of high red blood cell counts.

Treatment

The goal of treatment is to make the blood less thick and to prevent bleeding and clotting problems. Treatment options include:

  • Phlebotomy, which removes blood to make it less thick. One unit of blood, about 1 pint, is removed each week until the number of red blood cells drops. It is continued as needed.
  • Hydroxyurea, a medicine that lowers the number of red blood cells the bone marrow makes.
  • Interferons, medicines that lower blood counts, especially in pregnancy.
  • Ruxolitinib (Jakafi), a medicine that lowers red blood cells and shrinks an enlarged spleen. It is prescribed when hydroxyurea and other treatments have failed.
  • Aspirin, which may lower the chance of blood clots for some people. It also raises the risk of stomach bleeding.
  • Ultraviolet light therapy, which can ease severe itching.

When to see a doctor

Contact your provider if you develop symptoms of PV.

Call 911 or go to the emergency room if you have chest pain or discomfort lasting two minutes or more, trouble breathing, fainting, vomiting blood, severe belly pain, not being able to speak, or sudden dizziness, weakness, or a sudden change in vision. Clots from PV can cause a stroke or heart attack.

How a direct primary care or concierge doctor fits in

A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Either can review your symptoms over time, order routine blood tests, and help coordinate care with a hematologist.

Common questions

Can polycythemia vera be inherited?

Most of the time, no. Most cases are not inherited, because the gene changes are acquired during a person's lifetime. In rare cases, PV runs in families.

Does polycythemia vera cause blood clots?

Yes, it can. The extra blood cells make abnormal clots more likely to form and block blood flow in arteries and veins. This raises the risk of a heart attack or stroke.

What does phlebotomy do for polycythemia vera?

Phlebotomy lowers the thickness of the blood. A unit of blood is removed each week until the number of red blood cells drops, and it is continued as needed.

Does polycythemia vera cause itching?

Yes, it can. Itchiness is a symptom of PV, especially after a warm bath. Ultraviolet light therapy can ease severe itching.

Sources

  1. MedlinePlus - Polycythemia vera
  2. MedlinePlus Genetics - Polycythemia vera

Medical Disclaimer

This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.