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


Myelosuppressive Agents for the Treatment of Polycythemia Vera[4]

Agent Class Common side effects Uncommon side effects Percautions
Hydroxyurea (Hydrea) Antimetabolite Anemia, neutropenia, oral ulcers, skin ulcers, hyperpigmentation, nail changes Leg ulcers, nausea, diarrhea, fever, elevated liver function test results Renal disease
Recombinant interferon alfa-2b (Intron A) Myelosuppressive Influenza-like symptoms, fatigue, anorexia, weight loss, alopecia, headache, nausea, insomnia, body pain Confusion, depression, autoimmunity, hyperlipidemia Psychiatric disease, cardiovascular disease
Radioactive phosphorus Radiopharmaceutica Anemia, thrombocytopenia, leukopenia, Leukemia may develop after treatment Diarrhea, fever, nausea, emesis
Busulfan (Myleran) Alkylating agent Pancytopenia, hyperpigmentation, ovarian suppression Pulmonary fibrosis, leukemia, seizure, hepatic veno-occlusion Seizure disorder

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. 4.0 4.1 Stuart BJ, Viera AJ (2004). "Polycythemia vera". Am Fam Physician. 69 (9): 2139–44. PMID 15152961.

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