Essential thrombocythemia is a type of myeloproliferative neoplasm caused by the clonal proliferation of hematopoietic stem cells. It is characterized by a persistent increase in platelet count in peripheral blood, excessive proliferation of megakaryocytes in the bone marrow, and clinically, a tendency for thrombosis and spontaneous bleeding. It has an incidence rate of approximately 0.84 per 100,000 people per year.
Causes and Symptoms
The exact cause of Essential Thrombocythemia is not yet fully understood. In 60% of cases, mutations in the JAK2 gene are found, while 20-35% have CALR mutations, and 1-4% have MPL gene mutations. These genetic mutations are not hereditary but are acquired, and the exact cause of the mutations is not known. However, there are also patients with Essential Thrombocythemia who do not have these genetic mutations.
Treatment
The goal of treatment is to alleviate symptoms, prevent thrombosis, and prevent progression to myelofibrosis or acute leukemia. Essential thrombocythemia has two major complications: the first is thrombosis and bleeding, and the second is progression to acute leukemia and myelofibrosis. As the prevention of complications is closely tied to the patient's survival rate and quality of life, it is paramount to manage the cell count while conducting regular follow-up observations.
Treatment for Low-Risk Group
Aspirin
If there are cardiovascular risk factors or a JAK gene mutation, low-dose aspirin is administered to inhibit the aggregation of platelets and the formation of thrombus.
Treatment for High-Risk Group (In cases where the patient is over 60 years of age and has a JAK gene mutation, or has a history of thrombosis)
