Polycythemia vera

What is polycythemia vera?

Polycythemia vera (or primary) is a chronic condition characterized by an abnormality of the bone marrow cells, which generate too many red blood cells in the blood.

Among the consequences of this excess of red blood cells is that the blood is more viscous than normal, which makes its flow in the minor blood vessels more difficult. Polycythemia can therefore carry a high risk of developing blood clots and thrombosis.

It is an idiopathic disease, the cause of which is unknown. It affects approximately 2 persons out of every 100,000; it usually manifests itself around the age of 20 years, and affects men and women equally, although the male population is slightly more affected by this pathology.

Prognosis of the disease

Polycythemia vera is potentially very dangerous, as it promotes thrombosis and embolism, and may also facilitate the onset of leukemia.

It is estimated that the survival of people who do not adequately treat polycythemia vera is about 24 months. It is therefore essential that people suffering from polycythemia vera make frequent medical visits and scrupulously follow the hematology specialist’s instructions.

The disease generates too many red blood cells.

Symptoms of polycythemia vera

After an asymptomatic period, which can be more or less long, the first symptoms that appear are the following:

  • Weakness and tiredness;
  • Headache and headache;
  • Confusion and lightheadedness;
  • Shortness of breath;
  • Sweating during sleep;
  • Mild fever;
  • Typical symptoms indicating the presence of blood clots, such as localized swelling and pain in the affected area, and visibility of the vein below the surface of the skin.
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Diagnosis of polycythemia vera

During an objective examination, the specialist collects data on the possible enlargement of the spleen and alterations in breathing, vascular and cardiovascular circulation, renal, hepatic, neurological, ocular and gastrointestinal functions.

In addition, it is necessary to undergo a blood test to confirm that the number of red blood cells is too high.

A blood count and bone marrow examination may also be requested.

What are the causes of true polycythemia?

Although the triggering cause of polycythemia vera is not known, it has been observed that almost all patients affected by polycythemia vera have a mutation of a gene (JAK2) that causes hyperproduction of blood cells.

In rare cases, the transmission of the disease is genetic; in this case, it is called primitive familial polycythemia.

Treatments for polycythemia vera

The most common treatments for polycythemia vera are:

  • Drawing blood by phlebotomy: this consists of drawing approximately half a liter of blood every two days until normal values are reached; the treatment will be repeated after a few months if necessary;
  • Intake of drugs to reduce the number of red blood cells in the blood.

The aim of the therapy is to delay the evolution of polycythemia vera, but it cannot lead to a definitive cure.

Which specialist should be consulted?

The hematology specialist will be able to guide the patient through the diagnostic and therapeutic procedure for the treatment of polycythemia vera.