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

Peginterferon alfa-2a (Pegasys)

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Regimen

Study	Evidence
Kiladjian et al. 2008 (PVN1)	Phase II
Quintás-Cardama et al. 2009	Phase II

Quintás-Cardama et al. 2009 do not provide guidance on dose escalation or target dosing. Kiladjian et al. 2008 do not provide details on the dose escalation procedure.

• Peginterferon alfa-2a (Pegasys) 90 mcg SC once per week, titrated up every two weeks in the absence of toxicity to 135 mg SC once per week
  • In the absence of hematologic response, dose increased to 180 mg SC once per week

Supportive medications:

• Aspirin 100 mg PO once per day unless intolerant (Kiladjian et al. 2008)

Unspecified duration

References

1. Kiladjian JJ, Cassinat B, Chevret S, Turlure P, Cambier N, Roussel M, Bellucci S, Grandchamp B, Chomienne C, Fenaux P. Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera. Blood. 2008 Oct 15;112(8):3065-72. Epub 2008 Jul 23. link to original article contains verified protocol PubMed
2. Quintás-Cardama A, Kantarjian H, Manshouri T, Luthra R, Estrov Z, Pierce S, Richie MA, Borthakur G, Konopleva M, Cortes J, Verstovsek S. Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera. J Clin Oncol. 2009 Nov 10;27(32):5418-24. Epub 2009 Oct 13. link to original article contains protocol PubMed
   1. Update: Quintás-Cardama A, Abdel-Wahab O, Manshouri T, Kilpivaara O, Cortes J, Roupie AL, Zhang SJ, Harris D, Estrov Z, Kantarjian H, Levine RL, Verstovsek S. Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a. Blood. 2013 Aug 8;122(6):893-901. Epub 2013 Jun 19. link to original article PubMed

Ruxolitinib (Jakafi)

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Regimen

Study	Evidence	Comparator	Efficacy
Verstovsek et al. 2014	Phase II
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
   1. Update: Abstract: Jean-Jacques Kiladjian, Alessandro M. Vannucchi, Martin Griesshammer, Tamas Masszi, Simon Durrant, Francesco Passamonti, Claire N. Harrison, Fabrizio Pane, Pierre Zachee, Keita Kirito, Mario Cazzola, Carlos Besses, Igor Blau, Ruben Mesa, Mark M. Jones, Shui He, Huiling Zhen, Jingjin Li, Nathalie Francillard, Dany Habr, Srdan Verstovsek. Ruxolitinib versus best available therapy in patients with polycythemia vera: 80-week follow-up from the response trial. EHA 2015 Abstract: S447 link to abstract