Polycythemia vera

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10 regimens on this page 11 variants on this page

Relapsed, refractory, or intolerant

Anagrelide (Agrylin)

Regimen

Study	Evidence	Comparator	Efficacy
Vannucchi et al. 2015 (RESPONSE)	Phase III	Ruxolitinib	Decreased disease control

To be completed

References

1. Vannucchi AM, Kiladjian JJ, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Mesa R, He S, Jones MM, Garrett W, Li J, Pirron U, Habr D, Verstovsek S. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015 Jan 29;372(5):426-35. link to original article contains verified protocol PubMed

Hydroxyurea (Hydrea)

Regimen

Study	Evidence	Comparator	Efficacy
Vannucchi et al. 2015 (RESPONSE)	Phase III	Ruxolitinib	Decreased disease control

The following is the approach recommended by Vannucchi in How I Treat (2014).

• Hydroxyurea (Hydrea) 500 mg PO BID, with titration based on the target hematocrit and hematologic toxicity

References

1. Vannucchi AM. How I treat polycythemia vera. Blood. 2014 Nov 20;124(22):3212-20. Epub 2014 Oct 2. link to original article PubMed
2. Vannucchi AM, Kiladjian JJ, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Mesa R, He S, Jones MM, Garrett W, Li J, Pirron U, Habr D, Verstovsek S. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015 Jan 29;372(5):426-35. link to original article contains verified protocol PubMed

Ruxolitinib (Jakafi)

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Regimen

Study	Evidence	Comparator	Efficacy
Verstovsek et al. 2014	Phase II	N/A	N/A
Vannucchi et al. 2015 (RESPONSE)	Phase III	Standard therapy	Increased disease control

There were several doses evaluated in Verstovsek et al. 2014; the median total daily dose was 21.7 mg corresponding to approximately 10 mg PO twice per day. The most common comparator in RESPONSE was hydroxyurea, followed by interferon and anagrelide.

• Ruxolitinib (Jakafi) 10 mg PO twice per day

Continued until progression or excess toxicity

References

1. Verstovsek S, Passamonti F, Rambaldi A, Barosi G, Rosen PJ, Rumi E, Gattoni E, Pieri L, Guglielmelli P, Elena C, He S, Contel N, Mookerjee B, Sandor V, Cazzola M, Kantarjian HM, Barbui T, Vannucchi AM. A phase 2 study of ruxolitinib, an oral JAK1 and JAK2 Inhibitor, in patients with advanced polycythemia vera who are refractory or intolerant to hydroxyurea. Cancer. 2014 Feb 15;120(4):513-20. link to original article contains verified protocol PubMed content property of HemOnc.org
2. Vannucchi AM, Kiladjian JJ, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Mesa R, He S, Jones MM, Garrett W, Li J, Pirron U, Habr D, Verstovsek S. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015 Jan 29;372(5):426-35. link to original article contains verified protocol PubMed