Polycythemia vera


Polycythemia vera (PV) is a Rare Disease of the blood-building cells in the bone marrow primarily resulting in a chronic increase of red blood cells (erythrocytes), thereby decreasing the flowability of blood. Circulatory disorders such as thrombosis and embolism are possible consequences.
With proper treatment, the majority of patients with polycythemia vera have a near-normal life expectancy.

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    Cause & Symptoms
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    Diagnosis & Therapy

Diagnosis

The patient will be referred to a specialist (hematologist) for further examination if polycythemia vera is suspected (due to unspecified symptoms and a change in the blood count such as an increase in hemoglobin or hematocrit).

During the diagnosis it is important to first check if the increase in erythrocytes does not stem from a reactive condition caused by an underlying disease. Hence, underlying conditions in the heart, lungs, liver or kidney need to be ruled out as possible causes.

Blood analysis

The number of erythrocytes, together with the hemoglobin and hematocrit results, are elevated (hemoglobin: blood pigment, hematocrit: cellular components of blood).

An increase in thrombocytes and leucocytes also points to the existence of PV.

Genetic analysis

95% of all PV patients are carriers of the JAK2V617F mutation.

Measurement of the erythropoietin levels in the blood

If the kidneys suffer from a lack of oxygen, they release erythropoietin which stimulates the production of blood cells in the bone marrow (erythropoiesis). However, in the case of polycythemia vera, a rise in the erythrocyte count provides the organ with a higher amount of oxygen, which leads to a decline in the production of erythropoietin.

Ultrasound scan

The upper abdomen should undergo an ultrasound scan in order to check the size of the liver and spleen. These organs are sometimes swollen in patients with polycythemia vera.

Bone marrow puncture

The removal and histological examination of bone marrow from the iliac crest region may provide verification in uncertain cases.

Treatment

There is currently no cure for the disease. The treatment for the disease focuses on providing relief from the symptoms, preventing of blood clots and slowing the progression of the disease.

  • Regular phlebotomy (bloodletting)
    The goal of this treatment is to reduce the erythrocyte count in order to minimize the risk of a thrombosis. This can be accomplished in the early stages of the disease by undergoing periodic bloodletting. The removed blood is replaced by an electrolyte solution and plasma. However a simultaneous increase in thrombocytes and leukocytes cannot be controlled through this procedure.
  • Acetylsalicylic acid
    Acetylsalicylic acid is a known painkiller that is frequently administered when the thrombocyte count is too high in order to stop platelets from clogging (platelet aggregation). However, this treatment cannot reduce the thrombocyte count.
  • Controlling blood cell count using drugs (cytoreduction)
    Cytoreduction is performed in addition to other treatment for patients who have a high cardiovascular risk (Age >60 years or/and have a history of blood clots)
  • Hydroxyurea
    Older patients can choose hydroxyurea for nonspecific cytoreductive treatment.
  • Interferon alpha
    Interferon alpha is being used increasingly among younger patients (<60 years)in an effort to induce a more profound disease remission during long-term treatment.
  • Anagrelide
    Anagrelide (in many central European countries it is known as thromboreductin) is sometimes prescribed as an add-on therapy, when first-line PV therapy may not adequately control the number of thrombocytes (platelets).