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MPNs are usually not preventable or curable. The goal of MPN 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.
CML is treated with a specialised small molecule drugs called tyrosine kinase inhibitor which bind specifically to the BCR-ABL tyrosine kinase and stop it working. Imatinib (also known by its trade name as Glivec®) is the drug that most patients are started on when they are diagnosed with CML. Measurements of the BCR/ABL gene with a blood test are used to monitor the effectiveness of therapy. These drugs have been used in routine treatment since the late 1990’s early 2000’s. It is still too early to tell but there is hope that at least some patients will be cured with this therapy.
With polycythaemia vera (PV), frequent venesections, the removal of units of blood, are used to decrease the number of RBCs and to decrease the blood volume (haematocrit). Once RBCs have been lowered as close to normal limits as possible, the person is monitored, and occasional venesection 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.
The risk of thrombotic complications such as strokes, heart disease and clots in the body is usually managed with aspirin in patients with both PV and ET. If patients have a history of clots or stroke they may need management with other anticoagulant medication such as warfarin.
In addition to venesections, hydroxyurea, a chemotherapy drug, may be given to decrease the number of cells produced in both ET and PV. 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 anaemia, and surgical removal of the spleen may be required if it becomes too swollen (this may happen with any of the MPNs).
Last Review Date: January 13, 2020