Myeloproliferative Disorders
MPDs are usually not preventable or curable. The goals of MPD treatment are 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 and volume of RBCs in the blood. 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 itching may persist and the person may develop symptoms of iron deficiency.
In addition to phlebotomies, hydroxyurea, a chemotherapy drug, may be given to decrease the number of cells produced. Hydroxyurea or radiation may also be used when someone has myelofibrosis to help temporarily reduce splenomegaly pain, but this may also decrease the number of WBCs, leaving the person more vulnerable to infection. If a suitable donor is available, a bone marrow transplant may offer a potential cure for myelofibrosis in some younger patients.
Frequent blood transfusions may 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). 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 hydroxyurea 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 During this procedure, blood is withdrawn, the platelets are removed, and then the platelet-depleted blood is returned.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 may return platelet levels to normal.
