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Alternative Names Return to topEssential thrombocythemia; Essential thrombocytosis
Definition Return to top
Primary thrombocythemia is the overproduction of platelets without a known cause. Platelets are essential for blood clotting.
Causes Return to top
Primary thrombocythemia is a disorder that slowly progresses. It is caused by too much growth of a type of cell that is used to make blood cells. Although the platelets are mostly affected, the red blood cells and white blood cells may also be involved.
The disease is in the same family of disorders as:
Usually it affects people in middle age. It can also be seen in younger patients, especially females under age 40.
People with this condition make too many platelets and may have a problem with blood clots (thrombosis). Because these platelets do not work well, bleeding is also a common problem.
Symptoms Return to top
The condition may even cause strokes in some people.
Exams and Tests Return to top
This condition is often detected on blood tests done for other reasons, before there are any symptoms.
A physical exam may show an enlarged spleen or liver.
Other tests may include:
Treatment Return to top
If a patient has life-threatening complications, a procedure to remove platelets directly from the blood (platelet pheresis) can rapidly decrease the platelet count.
Long-term use of medications to decrease the platelet count can reduce both bleeding and clotting complications. The most commonly used medications include hydroxyurea, interferon-alpha, or anagrelide. In patients who have a tendency to clot, aspirin may help decrease clotting episodes.
Many patients do not need any treatment. However, the health care provider should monitor their condition.
Outlook (Prognosis) Return to top
The outcome varies. Most people go long periods of time without complications and have a normal life expectancy. In a very small minority of patients, complications from hemorrhage and thrombosis lead to death.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider:
References Return to top
Tefferi A. Myeloproliferative disorders: Essential thrombocythemia and primary myelofibrosis. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 177.Update Date: 3/2/2009 Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.