Polycythemia Vera Medication

Updated: Dec 02, 2016
  • Author: Srikanth Nagalla, MBBS, MS, FACP; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Medication

Medication Summary

One objective of therapy for polycythemia vera (PV) is to control the myeloproliferative activity of this disease. Evidence of an increase in levels of white blood cells and/or platelets and organomegaly indicate uncontrolled myeloproliferative activity that requires a myelosuppressive agent.

Studies by the Polycythemia Vera Study Group (PVSG) have led to the abandonment of long-term therapy with phosphorus-32 (32P) and most alkylating agents (eg, busulfan, chlorambucil), and to the use of hydroxyurea (HU) instead. However, long-term data seem to indicate a possible slight late increase in cases of acute leukemia in patients with PV who are treated with HU for more than 15 years.

Ruxolitinib is now approved in the United States for intermediate- or high-risk myelofibrosis, including primary myelofibrosis, post-PV myelofibrosis, and post–essential thrombocythemia myelofibrosis.

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Antimetabolites

Class Summary

HU is a nonalkylating agent that inhibits DNA synthesis and cell replication by blocking the enzyme ribonucleoside diphosphate reductase.

Hydroxyurea (Droxia, Hydrea)

Inhibitor of deoxynucleotide synthesis and DOC for inducing hematologic remission in CML. Less leukemogenic than alkylating agents such as busulfan, melphalan, or chlorambucil. Myelosuppressive effects last a few days to a week and are easier to control than those of alkylating agents; busulfan has prolonged marrow suppression and can cause pulmonary fibrosis. Can be administered at higher doses in patients with extremely high WBC counts (>300,000/µL) and adjusted accordingly as counts fall and platelet counts drop. Dose can be administered as a single daily dose or divided into 2-3 doses at higher dose ranges. Droxia, available in smaller tabs of 200, 300, and 400 mg, is for patients with sickle cell disease.

Ruxolitinib (Jakafi)

JAK1/JAK2 kinase inhibitor indicated for polycythemia vera in patients who have had an inadequate response to or are intolerant of hydroxyurea. Janus-associated kinases (JAKs) JAK1 and JAK2 mediate the signaling of a number of cytokines and growth factors that are important for hematopoiesis and immune function.

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Imidazole Quinazolines

Class Summary

Imidazole quinazolines have been demonstrated to have powerful anti-aggregating effects on platelets and to cause thrombocytopenia.

Anagrelide hydrochloride (Agrylin)

Primary activity is to lower platelet levels but shows slight decrease in mean hemoglobin and hematocrit while WBC counts maintained. Effective in polycythemia vera with elevated platelet counts. Adjust dosage to lowest effective dose to reduce and maintain platelet counts, WBC count, and hemoglobin levels within reference range.

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Interferons

Class Summary

Recombinant interferon alfa is a biologic response modifier with myelosuppressive activity.

Interferon alfa 2b (Interferon alfa-2b, Intron A)

Protein product manufactured by recombinant DNA technology. Can lower counts and shrink enlarged spleens.

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