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Genetic and Rare Diseases Information Center (GARD)

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Essential thrombocythemia

Other Names for this Disease
  • Essential thrombocytosis
  • Hemorrhagic thrombocythemia
  • Idiopathic thrombocythemia
  • Primary thrombocythemia
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Your Question

What causes essential thrombocythemia? How long do you live with it? What was the oldest that live with the disorder?

Our Answer

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What is essential thrombocythemia?

Essential thrombocythemia belongs to a group of conditions called myeloproliferative disorders. Myeloproliferative disorders cause platelets, white blood cells and red blood cells to grow abnormally in the bone marrow (the soft tissue inside the hollow part of bones that helps form blood cells). In essential thrombocythemia, the body produces too many platelet cells. The signs and symptoms vary from person to person, with up to two-thirds of patients not having any symptoms when the platelet cell count first increases. Signs and symptoms may include significant increased production of megakaryocyte (a cell in the bone marrow that is responsible for making platelets), enlargement of the spleen (splenomegaly), and bleeding and/or clotting episodes.[1][2]
Last updated: 6/30/2011

What causes essential thrombocythemia?

In this condition, faulty stem cells in the bone marrow make too many abnormal platelets, which causes blood clots (thrombosis) and sometimes bleeding. What causes this to happen usually isn't known. Rarely, essential thrombocythemia is inherited. In some cases, a genetic mutation may cause the condition.[1][2] 
Last updated: 6/30/2011

How long do you live with essential thrombocythemia?

Because the symptoms seen in patients with essential thrombocytosis vary from person to person, the prognosis is also different from person to person. In general, most people go long periods of time without complications and have a normal life expectancy. In a very small number of patients, life-threatening complications arise from a bleeding or clotting episode or from transformation into either primary myelofibrosis or acute myeloid leukemia.[1]
Last updated: 6/30/2011

What was the oldest that a person with essential thrombocythemia has lived?

Because this type of information is not tracked and because some people with essential thrombocythemia do not present with symptoms and are therefore not diagnosed, it is unknown the age of the oldest person with essential thrombocythemia. Essential thrombocythemia usually affects people in middle age. It can also be seen in younger patients, especially females under age 40.[1]
Last updated: 6/30/2011