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Essential thrombocythemia: past and present.
Fabrizio Fabris1, Maria Luigia Randi
1Department of Medical and Surgical Sciences, University of Padua Medical School, Via Giustiniani 2, 35128 Padua, Italy. fabrizio.fabris@unipd.it
Internal and Emergency Medicine
|July 29, 2009
Summary
Essential thrombocythemia (ET) is a blood disorder causing increased platelets and clot risk. JAK2 mutations are common, and while treatments exist, thrombosis remains a key concern for patients.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by elevated platelet counts.
- It is associated with an increased risk of thromboembolic events and microvascular disturbances.
Observation:
- A significant proportion of ET patients harbor a JAK2 gene mutation (JAK2V617F).
- ET predominantly affects women, with a median diagnosis age of 65-70 years, though it can occur in all age groups.
- Increased miscarriage rates are observed in women with ET, particularly those with the JAK2V617F mutation.
Findings:
- Major thrombosis, arterial or venous, is the primary cause of morbidity and mortality in ET patients.
- Risk factors for thrombosis include age over 60 and a history of prior thrombosis.
- Hemorrhages are less common but can occur in patients with extremely high platelet counts (>1,500 x 10(9)/L).
- Progression to myelofibrosis and leukemia is more frequent in JAK2V617F-positive individuals.
Implications:
- Low-dose aspirin can help manage microvascular symptoms and prevent thrombosis in high-risk ET patients.
- Hydroxyurea is a primary treatment for high-risk ET patients, with other agents available for management.
- Understanding ET's genetic basis and risk factors is crucial for optimizing patient care and reducing complications.
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