Polycythemia vera, a myeloproliferative neoplasm, results from a genetic mutation which leads to the production of too many red blood cells (erythrocytosis).The white blood cell and platelet counts may also be elevated. This increase in the number of blood cells (without an increase in the liquid portion of blood) increases the risk of clot development.
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Learning you have beendiagnosed with polycythemia veracan be distressing and naturally, you may wonder, “How can it be treated?” Fortunately,with treatment, survival increasesfrom six to 18 months to 13 years or more. One of the challenges in treating polycythemia vera is that more than 12 percent of people will transform intomyelofibrosisand around 7 percent will develop acute leukemia/myelodysplastic syndrome.
In contrast to most people withessential thrombocythemia, people with polycythemia veracommonly have symptoms likeheadache, dizziness, or itchiness at diagnosis. These symptoms are generally distressing and require the initiation of treatment.
Phlebotomy for PV
Hydroxyurea for PV
In people with a high risk of developing a blood clot (more than 60 years of age, history of blood clots), additional therapy is needed.Often the first medication added to therapeutic phlebotomy is oral hydroxyurea.
Low-dose aspirin (81– 100 mg/d) is recommended as a front-line therapy for:Patients with low-risk PV (those age 60 and under with no history of thrombosis). Phlebotomy is also recommended.Patients with high-risk PV (those over the age of 60 and/or a history of thrombosis). Both phlebotomy and cytoreductive therapy are also recommended.
Low-dose aspirin (81– 100 mg/d) is recommended as a front-line therapy for:
Hydroxyurea is an oral chemotherapeutic agent that reduces the production of red blood cells in the bone marrow. It also reduces the production of white blood cells and platelets. Similar to other conditions treated with hydroxyurea, it is started at a low dose and increased until the hematocrit is in the goal range.
It is normally well tolerated with few side effects other than the decreased production of blood cells. Other side effects include oral ulcers, very sore throat, fatigue, weakness, bruising, wounds that won’t heal, and more rarely there can be hyperpigmentation (dark discoloration of the skin), and nail changes (dark lines under nails).
Interferon Alpha for PV
For people who have PV symptoms such as unmanageable itching (called pruritus) and enlarged spleen, interferon-alpha may be used. It is usually reserved for younger, more physically fit patients, as well as women who are pregnant or in childbearing years, or refractory to prior therapies.Interferon-alpha is a subcutaneous injection that reduces the red blood cell count. It can also reduce spleen size and itching. Side effects include fever, weakness, nausea, and vomiting which limits the usefulness of this medication.
Busulfan for PV
For patients that have failed hydroxyurea and/or interferon-alpha, busulfan, a chemotherapeutic medication, may be used.The dose is changed to keep the white blood cell count and platelet count in an acceptable range.
However, Busulfan has fallen out of favor for the treatment of PV due to potential side effects that include low blood counts, pulmonary fibrosis, and hyperpigmentation.
Ruxolitinib for PV
Another medication used for patients that failed other therapies isruxolitinib. This medication inhibits the JAK enzyme that is often affected in polycythemia vera and other myeloproliferative neoplasms.It is often used for people who develop post-polycythemia vera myelofibrosis. It can also be helpful in people with severe symptoms and splenomegaly (enlarged spleen) that causes significant pain or other issues.
Remember, although polycythemia vera is a chronic condition, it is manageable. Talk to your healthcare provider about the various treatment options available.
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10 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Raedler LA.Diagnosis and management of polycythemia vera: Proceedings from a multidisciplinary roundtable.Am Health Drug Benefits. 2014;7(7 Suppl 3):S36-47.Gotlib J.In polycythemia vera, 45 is the number.The Hematologist. 2013;10(2). doi:10.1182/hem.V10.2.1077Ginzburg YZ, Feola M, Zimran E, Varkonyi J, Ganz T, Hoffman R.Dysregulated iron metabolism in polycythemia vera: Etiology and consequences.Leukemia. 2018;32(10):2105-2116. doi:10.1038/s41375-018-0207-9Büyükaşık Y, Ali R, Turgut M, et al.Patterns of hydroxyurea prescription and use in routine clinical management of polycythaemia vera: A multicenter chart review study.Turk J Hematol. 2020;37:177-185. doi:10.4274/tjh.galenos.2020.2019.0431Benevolo G, Vassallo F, Urbino I, Giai V.Polycythemia vera (PV): Update on emerging treatment options.Ther Clin Risk Manag. 2021;17:209-221. doi:10.2147/TCRM.S213020Karanth S, Gupta A, Prabhu M.Melanonychia and mucocutaneous hyperpigmentation from hydroxyurea use for the treatment of essential thrombocytosis.Singapore Med J. 2014;55(1):e7-e8. doi:10.11622/smedj.2013187Zhang M, Fu S, Ren D, et al.Maternal and fetal outcomes after interferon exposure during pregnancy: A systematic review with meta-analysis.Front Reprod Health. 2021;3. doi:10.3389/frph.2021.702929Begna K, Abdelatif A, Schwager S, Hanson C, Pardanani A, Tefferi A.Busulfan for the treatment of myeloproliferative neoplasms: The Mayo Clinic experience.Blood Cancer Journal. 2016;6(5):e427-e427. doi:10.1038%2Fbcj.2016.34Cancer Research UK.Busulfan.Geyer HL, Mesa RA.Therapy for myeloproliferative neoplasms: When, which agent, and how?Blood. 2014;124(24):3529-3537. doi:10.1182/blood-2014-05-577635
10 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Raedler LA.Diagnosis and management of polycythemia vera: Proceedings from a multidisciplinary roundtable.Am Health Drug Benefits. 2014;7(7 Suppl 3):S36-47.Gotlib J.In polycythemia vera, 45 is the number.The Hematologist. 2013;10(2). doi:10.1182/hem.V10.2.1077Ginzburg YZ, Feola M, Zimran E, Varkonyi J, Ganz T, Hoffman R.Dysregulated iron metabolism in polycythemia vera: Etiology and consequences.Leukemia. 2018;32(10):2105-2116. doi:10.1038/s41375-018-0207-9Büyükaşık Y, Ali R, Turgut M, et al.Patterns of hydroxyurea prescription and use in routine clinical management of polycythaemia vera: A multicenter chart review study.Turk J Hematol. 2020;37:177-185. doi:10.4274/tjh.galenos.2020.2019.0431Benevolo G, Vassallo F, Urbino I, Giai V.Polycythemia vera (PV): Update on emerging treatment options.Ther Clin Risk Manag. 2021;17:209-221. doi:10.2147/TCRM.S213020Karanth S, Gupta A, Prabhu M.Melanonychia and mucocutaneous hyperpigmentation from hydroxyurea use for the treatment of essential thrombocytosis.Singapore Med J. 2014;55(1):e7-e8. doi:10.11622/smedj.2013187Zhang M, Fu S, Ren D, et al.Maternal and fetal outcomes after interferon exposure during pregnancy: A systematic review with meta-analysis.Front Reprod Health. 2021;3. doi:10.3389/frph.2021.702929Begna K, Abdelatif A, Schwager S, Hanson C, Pardanani A, Tefferi A.Busulfan for the treatment of myeloproliferative neoplasms: The Mayo Clinic experience.Blood Cancer Journal. 2016;6(5):e427-e427. doi:10.1038%2Fbcj.2016.34Cancer Research UK.Busulfan.Geyer HL, Mesa RA.Therapy for myeloproliferative neoplasms: When, which agent, and how?Blood. 2014;124(24):3529-3537. doi:10.1182/blood-2014-05-577635
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Raedler LA.Diagnosis and management of polycythemia vera: Proceedings from a multidisciplinary roundtable.Am Health Drug Benefits. 2014;7(7 Suppl 3):S36-47.Gotlib J.In polycythemia vera, 45 is the number.The Hematologist. 2013;10(2). doi:10.1182/hem.V10.2.1077Ginzburg YZ, Feola M, Zimran E, Varkonyi J, Ganz T, Hoffman R.Dysregulated iron metabolism in polycythemia vera: Etiology and consequences.Leukemia. 2018;32(10):2105-2116. doi:10.1038/s41375-018-0207-9Büyükaşık Y, Ali R, Turgut M, et al.Patterns of hydroxyurea prescription and use in routine clinical management of polycythaemia vera: A multicenter chart review study.Turk J Hematol. 2020;37:177-185. doi:10.4274/tjh.galenos.2020.2019.0431Benevolo G, Vassallo F, Urbino I, Giai V.Polycythemia vera (PV): Update on emerging treatment options.Ther Clin Risk Manag. 2021;17:209-221. doi:10.2147/TCRM.S213020Karanth S, Gupta A, Prabhu M.Melanonychia and mucocutaneous hyperpigmentation from hydroxyurea use for the treatment of essential thrombocytosis.Singapore Med J. 2014;55(1):e7-e8. doi:10.11622/smedj.2013187Zhang M, Fu S, Ren D, et al.Maternal and fetal outcomes after interferon exposure during pregnancy: A systematic review with meta-analysis.Front Reprod Health. 2021;3. doi:10.3389/frph.2021.702929Begna K, Abdelatif A, Schwager S, Hanson C, Pardanani A, Tefferi A.Busulfan for the treatment of myeloproliferative neoplasms: The Mayo Clinic experience.Blood Cancer Journal. 2016;6(5):e427-e427. doi:10.1038%2Fbcj.2016.34Cancer Research UK.Busulfan.Geyer HL, Mesa RA.Therapy for myeloproliferative neoplasms: When, which agent, and how?Blood. 2014;124(24):3529-3537. doi:10.1182/blood-2014-05-577635
Raedler LA.Diagnosis and management of polycythemia vera: Proceedings from a multidisciplinary roundtable.Am Health Drug Benefits. 2014;7(7 Suppl 3):S36-47.
Gotlib J.In polycythemia vera, 45 is the number.The Hematologist. 2013;10(2). doi:10.1182/hem.V10.2.1077
Ginzburg YZ, Feola M, Zimran E, Varkonyi J, Ganz T, Hoffman R.Dysregulated iron metabolism in polycythemia vera: Etiology and consequences.Leukemia. 2018;32(10):2105-2116. doi:10.1038/s41375-018-0207-9
Büyükaşık Y, Ali R, Turgut M, et al.Patterns of hydroxyurea prescription and use in routine clinical management of polycythaemia vera: A multicenter chart review study.Turk J Hematol. 2020;37:177-185. doi:10.4274/tjh.galenos.2020.2019.0431
Benevolo G, Vassallo F, Urbino I, Giai V.Polycythemia vera (PV): Update on emerging treatment options.Ther Clin Risk Manag. 2021;17:209-221. doi:10.2147/TCRM.S213020
Karanth S, Gupta A, Prabhu M.Melanonychia and mucocutaneous hyperpigmentation from hydroxyurea use for the treatment of essential thrombocytosis.Singapore Med J. 2014;55(1):e7-e8. doi:10.11622/smedj.2013187
Zhang M, Fu S, Ren D, et al.Maternal and fetal outcomes after interferon exposure during pregnancy: A systematic review with meta-analysis.Front Reprod Health. 2021;3. doi:10.3389/frph.2021.702929
Begna K, Abdelatif A, Schwager S, Hanson C, Pardanani A, Tefferi A.Busulfan for the treatment of myeloproliferative neoplasms: The Mayo Clinic experience.Blood Cancer Journal. 2016;6(5):e427-e427. doi:10.1038%2Fbcj.2016.34
Cancer Research UK.Busulfan.
Geyer HL, Mesa RA.Therapy for myeloproliferative neoplasms: When, which agent, and how?Blood. 2014;124(24):3529-3537. doi:10.1182/blood-2014-05-577635
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