The optimal management of PV remains elusive despite the findings of the PVSG. However, general principles in the management of PV include the following:
Tailor therapy to suit the clinical needs of the patient; consider the status of the formed elements of the blood, bone marrow, and organomegaly.
Normalize red blood cell mass with phlebotomy as rapidly as clinically possible (250-500 mL every other day); patients who are elderly or have cardiovascular compromise should be phlebotomized cautiously, and smaller amounts should be removed.
Suppress myeloproliferative activity with chemotherapy (hydroxyurea) in all patients older than 50 years.
The most recent advance in the treatment of PV is the development of JAK inhibitors. The JAK1/2 inhibitor ruxolitinib is approved for treatment of PV in patients who have had an inadequate response to or are intolerant of hydroxyurea.
In general, 32P therapy should be reserved for patients older than 80 years or patients with comorbid conditions in whom life expectancy is less than 5-10 years and the convenience of 32P dosing outweighs the substantial risks of developing acute leukemia 5-15 years after 32P administration.
Patients with thrombotic tendencies or those who develop thrombocytosis after phlebotomy should be treated with marrow suppression; consider anagrelide in younger patients (aged 50-70 years).
Maintain blood values at reference range levels by regular examination and treatment.
Avoid overtreatment and toxicity by careful and judicious use of chemotherapy and radiation; supplemental phlebotomy is preferred over excess marrow suppression.
Postpone elective surgery until long-term control of the disease is established.
Women of childbearing age should be treated with phlebotomy only.
In young males, myelosuppressive therapy can lead to aspermia; thus, evaluate treatment carefully before using any chemotherapy or radiation therapy.
The PVSG no longer recommends the use of alkylating agents because of the associated increased incidence of leukemia and certain types of cancer.
Treat hyperuricemia with allopurinol (100-300 mg/day) until remission has been attained; for acute gouty attacks, colchicine or other anti-inflammatory agents are indicated.
For more on the treatment of PV, read here.
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Any views expressed above are the author's own and do not necessarily reflect the views of WebMD or Medscape.
Cite this: Emmanuel C. Besa. Fast Five Quiz: Are You Familiar With Key Aspects of Polycythemia Vera? - Medscape - Apr 04, 2018.