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Myeloproliferative Disorders (MPD)

Treatment
MPDs are usually not preventable or curable.  The goal of MPD treatment is to slow the progression of the disease and to alleviate the symptoms and complications brought on by excessive, insufficient, and dysfunctional blood cell production. With polycythemia vera, frequent phlebotomies (the removal of pints of blood) are used to decrease the number of RBCs and to decrease the blood volume (hematocrit).  Once RBCs have been lowered as close to normal limits as possible, the person is monitored, and occasional phlebotomies are used to keep the levels under control.  Splenomegaly (the enlargement of the spleen) and pruritis (itching) may persist and the patient may develop symptoms of iron deficiency.  If phlebotomy cannot be done, the patient may be given hydroxyurea (a chemotherapy drug) to decrease the number of cells produced.

Hydroxyurea (or radiation) may also be used when the patient has myelofibrosis, to help temporarily reduce splenomegaly pain, but this may also decrease the number of WBCs leaving the patient more vulnerable to infection and the need for frequent antibiotics.  Blood transfusions may often be necessary to address anemia, and surgical removal of the spleen may be required if it becomes too swollen (this may happen with any of the MPDs).  If a suitable donor is available, a bone marrow transplant may offer a potential cure for myelofibrosis in some younger patients.

Asymptomatic patients with thrombocythemia are monitored but they may or may not be treated, - there is not general agreement on its necessity.  Small doses of aspirin, which make platelets less sticky and slow clotting may suffice, or hydroxurea or anagrelide (an anticlotting drug) may be used to reduce the number of platelets.  If platelet numbers are not responding to drug treatment rapidly enough, plateletpheresis may also be done.  During this procedure blood is withdrawn, the platelets are removed, and then the platelet-depleted blood is returned.  If thrombocythemia is due to a non-marrow cause, addressing the underlying condition should return platelet levels to normal.



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This article last reviewed on June 27, 2005.
 
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