Polycythemia vera medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2] Shyam Patel [3]
Overview
The mainstay of therapy for polycythemia vera is phlebotomy, hydroxyurea (alone or with phlebotomy), interferon-alpha and pegylated interferon-alpha, chlorambucil, and low-dose aspirin.[1]
Medical Therapy
Medical therapy for polycythemia vera include:[1][2][3][4][5][6][7][8][9][10][11]
- Phlebotomy: Phlebotomy is a therapeutic procedure that involves withdrawal of blood from a patient's body with the goal of reducing red blood cell mass and hemoglobin.
- Aspirin: Aspirin is an irreversible inhibitor of cyclo-oxygenase (prostaglandin endoperoxide synthase) and thus inhibits for the formation of the prothrombotic molecule thromboxane A2. Low-dose aspirin of 81mg has been shown to help prevent thrombosis in patients with polycythemia vera.
- Hydroxyurea: Hydroxyurea inhibits ribonucleotide reductase, which is essential is nucleic acid metabolism. By inhibiting ribonucleotide reductase in hematopoietic cells, hydroxyurea results in decreased stem cell proliferation and decreased red blood cell mass. Adverse effects of hydroxyurea include cytopenias and skin ulcers.
- Interferon-alpha: This medication results in reduction in red blood cell mass. Adverse effects include infusion reaction and cytopenias. Pegylated interferon-alpha is one formulation of this medication.
- Chlorambucil or busulfan: These are alkylating agents that inhibit the proliferation of the malignant clone responsible for elevated red blood cell mass. Adverse effects include secondary myelodysplastic syndrome and secondary leukemias, which typically occur 5-7 years after exposure to these alkylating agents. These medications can be used if interferon or hydroxyurea are not tolerated.
References
- ↑ 1.0 1.1 National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_5
- ↑ Berk PD, Goldberg JD, Donovan PB, Fruchtman SM, Berlin NI, Wasserman LR (1986). "Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols". Semin Hematol. 23 (2): 132–43. PMID 3704665.
- ↑ Lamy T, Devillers A, Bernard M, Moisan A, Grulois I, Drenou B; et al. (1997). "Inapparent polycythemia vera: an unrecognized diagnosis". Am J Med. 102 (1): 14–20. PMID 9209196.
- ↑ Kaplan ME, Mack K, Goldberg JD, Donovan PB, Berk PD, Wasserman LR (1986). "Long-term management of polycythemia vera with hydroxyurea: a progress report". Semin Hematol. 23 (3): 167–71. PMID 3749925.
- ↑ Lengfelder E, Berger U, Hehlmann R (2000). "Interferon alpha in the treatment of polycythemia vera". Ann Hematol. 79 (3): 103–9. PMID 10803930.
- ↑ Silver RT (2006). "Long-term effects of the treatment of polycythemia vera with recombinant interferon-alpha". Cancer. 107 (3): 451–8. doi:10.1002/cncr.22026. PMID 16804923.
- ↑ Huang BT, Zeng QC, Zhao WH, Li BS, Chen RL (2014). "Interferon α-2b gains high sustained response therapy for advanced essential thrombocythemia and polycythemia vera with JAK2V617F positive mutation". Leuk Res. 38 (10): 1177–83. doi:10.1016/j.leukres.2014.06.019. PMID 25069759.
- ↑ Quintás-Cardama A, Kantarjian H, Manshouri T, Luthra R, Estrov Z, Pierce S; et al. (2009). "Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera". J Clin Oncol. 27 (32): 5418–24. doi:10.1200/JCO.2009.23.6075. PMID 19826111.
- ↑ Quintás-Cardama A, Abdel-Wahab O, Manshouri T, Kilpivaara O, Cortes J, Roupie AL; et al. (2013). "Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a". Blood. 122 (6): 893–901. doi:10.1182/blood-2012-07-442012. PMC 3739035. PMID 23782935.
- ↑ Finazzi G, Barbui T (2007). "How I treat patients with polycythemia vera". Blood. 109 (12): 5104–11. doi:10.1182/blood-2006-12-038968. PMID 17264301.
- ↑ Squizzato A, Romualdi E, Passamonti F, Middeldorp S (2013). "Antiplatelet drugs for polycythaemia vera and essential thrombocythaemia". Cochrane Database Syst Rev. 4: CD006503. doi:10.1002/14651858.CD006503.pub3. PMID 23633335.