Difference between revisions of "Polycythemia vera"
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Revision as of 14:21, 8 November 2015
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10 regimens on this page
11 variants on this page
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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
- 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
- Vannucchi AM. How I treat polycythemia vera. Blood. 2014 Nov 20;124(22):3212-20. Epub 2014 Oct 2. link to original article PubMed
- 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
- 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
- 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
- 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
- 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
- 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
- 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