Polycythemia medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Debduti Mukhopadhyay, M.B.B.S[2]

Overview

  • Phlebotomy: the backbone of therapy, treatment with phlebotomy alone associated with a longer median survival compared to the use of chlorambucil or radioactive phosphorous based on a trial conducted by PV study group. Repeated phlebotomies help in cytoreduction and reduce hyper-viscosity in addition to induce a state of iron-deficiency which can help retard red-cell proliferation. Weekly sessions are conducted, by remove 500ml of blood until a target hematocrit of under 45% is obtained. This can lower rates of cardiovascular deaths and major thrombotic episodes in patients kept under this threshold based on a trial conducted in Italy. For secondary polycythemias, phlebotomy is usually reserved for the following conditions:
    • Chronic lung diseases
    • Cyanotic heart diseases
    • Post-renal transplant patients with hypertension and erythrocytosis, not responding to optimal doses of angiotensin-converting enzyme inhibitors (ACEIs)/angiotensin receptor blockers (ARB) [1] [2]
  • Medicines that may be used include:[3]
    • Hydroxyurea: it used to reduce red blood cells counts and other blood cells if high. It also used to prevent thrombosis in patients >60 years of age with chronic PV.
    • Interferon: reduce blood cell counts.PegIFN can be used to reduce established splenomegaly but not usually to normal size.
    • Anagrelide: used to treat thrombocytosis.
    • Ruxolitinib (JAK1/2 inhibitor): proved to be effective in PPMF and chronic-phase PV; provided durable symptom relief, blood count control, and reduction in splenomegaly; and was superior to hydroxyurea.
    • Aspirin: used to prevent thrombosis.
  • Ultraviolet-B light therapy: reduce the itchiness.


Sumptomatic Treatment in Polycythemia Vera[4]

Symptoms Treatment
Pruritus H1 and H2 blocking antihistamines (diphenhydramine [Benadryl], cyproheptadine [Periactin], hydroxyzine [Atarax, Vistaril], fexofenadine [Allegra], terfenadine [Seldane])
Paroxetine (Paxil)
Oatmeal or starch baths (in lukewarm water)
Recombinant interferon alfa-2b (intronA)
Erythromelalgia Aspirin, 50 to 100 mg daily
Myelosuppressive agents

References

  1. Assi TB, Baz E (2014). "Current applications of therapeutic phlebotomy". Blood Transfus. 12 Suppl 1: s75–83. doi:10.2450/2013.0299-12. PMC 3934278. PMID 24120605.
  2. "StatPearls". 2020. PMID 30252337.
  3. Spivak JL (2019). "How I treat polycythemia vera". Blood. 134 (4): 341–352. doi:10.1182/blood.2018834044. PMID 31151982.
  4. Stuart BJ, Viera AJ (2004). "Polycythemia vera". Am Fam Physician. 69 (9): 2139–44. PMID 15152961.

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