The characteristic feature of essential (primary) thrombocythemia is a chronic increase in platelets in the blood. Doctors call this thrombocytosis.
Thrombocytes, also known as blood platelets, play a decisive role in blood clotting. In the event of injury, they attach themselves to the surrounding tissue, close the wound and ensure that the bleeding stops.
Thrombocythemia belongs to the group of malignant diseases of the haematopoietic system (myeloproliferative neoplasia) and occurs rather rarely.
In western industrialized countries, around 2.5 out of every 100,000 inhabitants develop the disease each year. Most patients are between 20 and 40 or between 60 and 70 years old. Women develop the disease twice as often as men.
Like all myeloproliferative diseases, thrombocythemia also has a genetic component. Due to a mutation in the genes of the blood-forming stem cells, these produce platelets in an uncontrolled manner.
In thrombocythemia , there is a significantly increased production of platelets (purple) @ MdBabul /AdobeStock
The main symptoms of the disease are a constant or slowly increasing increase in platelets in the blood.
The consequences are
The disturbed blood circulation in the fingers and toes leads to painful redness with swelling and burning. In this case, the doctor speaks of erythromelalgia.
The symptoms of thrombocythemia also include pain when walking due to a lack of blood flow to the legs, as well as concentration problems and visual disturbances.
Theliver and spleen break down the platelets after 5-12 days. Both organs can swell due to the increased number of thrombocytes. This hepatosplenomegaly is noticeable in those affected by pain or a feeling of pressure in the upper abdomen.
Although there is an increase in blood platelets that promote blood clotting, these are partially non-functional. In rare cases, and especially if there is a significant increase in platelets, this can lead to life-threatening bleeding.
Due to the increased viscosity of the blood, there is an increased risk of thromboembolic complications such as heart attack or stroke. These two events are the main causes of death in thrombocythemia.
It is not possible to cure the disease. Therapy should reduce the patient's symptoms and at the same time prevent complications.
First of all, the patient should take general measures to reduce the risk of thromboembolism.
These include
Anti-aggregating and cytoreductive therapy options are also used. Anti-aggregating treatment with aspirin is intended to prevent the formation of blood clots and vascular occlusion.
Cytoreductive therapy is carried out with interferon-alpha or hydroxyurea, among others. The aim is to reduce the number of thrombocytes in the blood.
Compared to other chronic myeloproliferative diseases, essential thrombocythemia has a fairly good prognosis. Those affected generally have a normal life expectancy. However, complications such as a heart attack or stroke can lead to severe disabilities.
Despite intensive research, thrombocythemia is still one of the diseases for which there is no curative drug therapy.
However, the doctor can support the patient in the individual course of the disease and reduce possible symptoms. A therapy that is tailored to the patient's individual situation can improve their quality of life.
Thrombocythemia is a malignant disease (cancer) of the hematopoietic system and is characterized by an increase in the number of platelets in the blood.
This thrombocytosis leads to circulatory disorders and/or bleeding. It is not possible to cure the disease, but drug therapies can help those affected to achieve a normal life expectancy.
