What is included in the treatment of polycythemia vera (PV)?

Updated: Aug 20, 2019
  • Author: Srikanth Nagalla, MBBS, MS, FACP; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Answer

The optimal management remains elusive despite the findings of the Polycythemia Vera Study Group (PVSG). [4] 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. [18]  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. [19]

  • In general, phosphorus-32 (32 P) 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 y).

  • 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 radiotherapy.

  • 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/d) until remission has been attained; for acute gouty attacks, colchicine or other anti-inflammatory agents are indicated (see Gout and Pseudogout)


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