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)

• Medicines that may be used include:
  • 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

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