Alternative Names: Essential thrombocythemia; Essential thrombocytosis

Causes, incidence, and risk factors:

Primary thrombocythemia is a�slowly progressing disorder�caused by overgrowth of a type of cell that is a precursor of blood cells. Although the platelets are primarily affected, the red blood cells and white blood cells are also involved.

The disease has similarities to polycythemia vera, chronic myelogenous leukemia, and myelofibrosis. Usually it affects people in middle age. Bleeding can occur from the gastrointestinal tract, respiratory system, urinary tract, or skin.

The formation of blood clots, called thrombosis, may coincide with bleeding episodes. It may even cause strokes in some people. Risk factors are unknown. The incidence is about 3 out of 100,000 people.

Symptoms:

• Nosebleeds (epistaxis )
• Bleeding from the gums
• Bleeding from the gastrointestinal tract
• Easy bruising
• Prolonged bleeding from surgical procedures or tooth extraction
• Enlarged lymph nodes (rare)
• Bloody stools
• Headache
• Numbness of hands or feet
• Dizziness
• Ulcers on fingers or toes

Signs and tests:

• Bone marrow aspiration.
• CBC that shows elevated platelet count.
• Uric acid level may be elevated.
• Physical examination may show enlarged spleen or liver.

Treatment:

If a patient is having life-threatening complications, rapid decrease of the platelet count may be achieved through platelet pheresis, a procedure to remove platelets from the blood directly.

Long-term decrease of the platelet count using medications can reduce both bleeding and clotting complications. Most common medications include hydroxyurea, interferon-alpha, or anagrelide. For patients with a known clotting tendency, aspirin may help decrease clotting episodes.

Some patients do not need any treatment.

Support Groups:

Expectations (prognosis):

The outcome varies with reports ranging from prolonged periods without complications in some people, to fatalities from complications related to hemorrhage and thrombosis in others.

Complications:

• Severe hemorrhage
• Thrombotic episodes (stroke, heart attack, or blood clots in extremities)
• Acute leukemia or myelofibrosis can develop in some patients

Calling your health care provider:

• If unexplained or prolonged bleeding occurs.
• If chest pain, leg pain, confusion, weakness, numbness, or other new symptoms develop.

Prevention: