Essential thrombocythemia (ET) is a myeloproliferative disease, i.e., it causes an uncontrolled increase in blood cells, and affects platelets in particular. The condition is classified as a cancer, as stated by Dr. Carmino Antonio Souza, hematologist at the Society of Medicine and Surgery of Campinas (SMCC), and is also known as hemorrhagic thrombocythemia.
Like polycythemia vera (PV), another myeloproliferative disease, ET has no single cause and may also be associated with the same JAK2 oncogene, but at a lower frequency than in PV. However, unlike PV, essential thrombocythemia has a higher incidence in younger adults, not in the elderly.
Read more:Polycythemia vera: what is the disease, symptoms and treatment
What are the symptoms?
According to the doctor, the most common symptom is cutaneous-mucosal bleeding, especially when the patient's platelet count gets too high.
In some cases, there may also be hyperviscosity, a condition that makes the blood "thickened" and unable to flow freely through the blood vessels.
Diagnosis of the disease can occur through a complete blood count, but there is also the possibility of the condition being identified after heavy bleeding that requires rapid intervention, as the hematologist explains.
Is essential thrombocythemia curable? What are the treatments?
The disease has no cure, but it is usually a perfectly manageable condition. "In general, intermittent treatment (when necessary) is done with the drug hydroxyurea," explains Dr. Carmino. "JAK2 oncogene inhibitors can be part of the treatment."
Furthermore, in urgent cases, it is also possible to perform a plateletpheresis procedure with mechanical removal of the "excess" platelets.
How long does a person with essential thrombocythemia live?
The doctor explains that the disease has a good prognosis, with good evolution in most cases. Usually, people who suffer from this condition die due to other causes.
However, in rare cases, essential thrombocythemia can kill, with progression to bone marrow fibrosis (with failure) and acute leukemia.
Also read: Low Platelets: What it is, causes and treatment
Source: Dr. Carmino Antonio Souza, hematologist of the Society of Medicine and Surgery of Campinas (SMCC) .