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Polycythemia: Symptoms & Treatment

Instructor: Artem Cheprasov

Artem has a doctor of veterinary medicine degree.

Too much of anything is a bad thing. You knew that. And in the case of having too many red blood cells, this holds true as well. Find out why as you learn about the symptoms and treatment of polycythemia vera.

What Is Polycythemia Vera?

We all know that not having enough red blood cells is a bad thing. You in fact have heard of this as anemia. People with anemia have inappropriate oxygen delivery to tissues because they lack enough red blood cells or the oxygen-carrying protein of red blood cells (called hemoglobin).

But what about the opposite? Is having too many blood cells, specifically red blood cells, a bad thing too? The condition that causes uncontrolled red blood cell production (and thus an increase in red blood cell mass within our bodies) is called polycythemia vera (PV). 'Poly-' means many, '-cythemia' means a condition of blood cells, and 'vera' means true.

While polycythemia leads to an increase in white blood cells and platelets as well, as you just learned, its most prominent feature is actually erythrocytosis, an increase in the production and total mass of red blood cells. This word come from 'erythro-', meaning red, and '-cytosis', which means a condition where there is more than a normal amount of cells.

Polycythemia vera is a chronic myeloproliferative disorder. Being chronic means the disorder is of a long duration; in fact, it may take decades to fully develop. It is also myeloproliferative, which means that the bone marrow makes too many blood cells way too quickly.

This is a blood smear from a patient with polycythemia vera. The larger cells are the white blood cells while the smaller, reddish-gray ones are the red blood cells.
Polycythemia vera

PV Symptoms

Understanding that the condition is myeloproliferative is important because many of the signs and symptoms of PV stem from this fact. Myeloproliferation results in a greater volume of blood, and it imparts a greater thickness to blood as well, neither of which is good.

Again, this is a progressive disorder, so it takes time to develop. At first there may be few signs or symptoms, if any. In fact, PV may be discovered through routine blood testing, such as during an annual exam. However, many other people may have signs and symptoms of PV early on.

These include:

  • Headaches
  • Memory problems
  • Tinnitus, or ringing in the ears
  • Problems with vision, like blurred vision
  • Fatigue, especially when exercising
  • Dyspnea, or shortness of breath
  • Hypertension, or high blood pressure
  • Pruritus (itching), especially after taking a bath
  • Bone pain
  • Pain from stomach ulcers
  • An enlarged spleen and/or liver
  • Bleeding easily and having nosebleeds

As PV progresses, people will develop blood clots within their blood vessels. In fact, this is one of the most important causes of death stemming from PV. In some instances, people will paradoxically develop anemia, the exact opposite of what PV actually is. Why? Well, the bone marrow is so exhausted by PV that it may eventually fail, perhaps permanently so. This means it won't be able to produce blood cells, and this leads to severe anemia. The exact mechanism behind this anemia isn't clear since some believe it has less to do with PV and more to do with perhaps another disease, a relative nutritional deficiency, or a treatment the patient is undergoing. This means the anemia may be reversible in some cases.

PV Treatments

Speaking of treatments, one of them is positively medieval. Have you ever heard how ancient and medieval people used to practice bloodletting to try to cure just about everything? Well, that stuff was a bunch of bologna. But in PV, bloodletting (technically called phlebotomy) is an actual treatment that involves the withdrawal of blood from a vein. This is done to bring down the number of blood cells and the blood volume at the same time.

Another form of treatment is generally called myelosuppression, which involves the use of medication to decrease the production of blood cells within the bone marrow. A medication called hydroxyurea may be used to that effect. Interferon alpha and radioactive phosphorus can also be used.

Phlebotomy and myelosuppression are the two core treatments for PV. However, other treatments that address the side effects of PV may be used as well, such as antihistamines to reduce itching and aspirin to reduce the risk of blood clots.

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