Primary thrombocythemia is a disorder that 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. This condition slowly gets worse over time.
The disease is in the same family of disorders as:
Genetic tests (to look for a change in the JAK2 gene)
Uric acid level
If a patient has life-threatening complications, a procedure to remove platelets directly from the blood (platelet pheresis) can quickly 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 are at high risk for clotting (older patients, those with very high platelet levels or who have had past clotting episodes), aspirin at a low dose (81 to 100 mg per day) decreases clotting episodes.
Many patients do not need any treatment. However, the health care provider should monitor their condition.
The outcome varies. Most people go long periods of time without complications and have a normal life expectancy. In a very small number of patients, complications from bleeding (hemorrhage) and blood clots (thrombosis) can cause serious problems.
In a small number of patients, the disease will change into acute leukemia or myelofibrosis.
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.