What is Essential Thrombocythemia (ET?)

Essential Thrombocythemia (ET) is a hematological malignancy classified in a group of bone marrow disorders known as Myeloproliferative Disorders (MPDs.) Included in this group are Polycythemia Vera (PV,) Chronic Myelogenous Leukemia (CML,) and Myelofibrosis (MF.) People with Essential Thrombocythemia have a bone marrow that produces too many platelets, making them susceptible to strokes and heart attacks. It can be life threatening if untreated. Currently there is no cure for Essential Thrombocythemia, and the disease can progress into a more serious form of a Myeloproliferative Disorder such as Myelofibrosis (MF) or Acute Myeloid Leukemia (AML.)

People with Essential Thrombocythemia may be asymptomatic at diagnosis and may not require treatment. Those who are symptomatic may present with symptoms related to small or large vessel thrombosis or minor bleeding. Some patients may suffer a mini-stroke (TIA) or heart attack due to a clot circulating in the blood. Clots in the small arteries of the toes and fingers are common leading to digital pain enhanced by warmth, distal gangrene and classic eythromelalgia.   The incidence of thrombotic and bleeding episodes is minimized but not eliminated with reduction of the platelet count to normal.