Essential thrombocytosis

Essential thrombocytosis (primary thrombocythemia) is a myeloproliferative disorder associated with an increase in number and size of circulating platelets.

Half of all patients are asymptomatic, but clinical presentations include thrombosis and bleeding. There are no pathognomonic features and it is a diagnosis of exclusion.

Low-risk asymptomatic patients do not need treatment. High-risk patients (e.g., those who are >60 years old, or those with a history of thrombosis or major bleeding) should be treated with aspirin and cytoreductive drugs. Treatment approach should be individualized based on risk factors.

Hydroxyurea is the preferred cytoreductive agent in most people with the disease. Interferon alfa-2b and busulfan are alternative treatments.

The life expectancy for people with essential thrombocytosis is usually similar to that for people without the disease. Patients need CBCs on a regular follow-up basis.

Essential thrombocytosis is also known as primary thrombocythemia. It is a chronic myeloproliferative disorder associated with sustained dysregulated megakaryocyte proliferation, increasing the number of circulating platelets. It is associated with thrombosis and bleeding.