Difference between revisions of "Polycythemia vera"
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− | '''Use of this site is subject to you reading and agreeing with the terms set forth in the [[HemOnc.org_-_A_Hematology_Oncology_Wiki:General_disclaimer|disclaimer]]. If this is your first time visiting, we suggest you read the [[tutorial]].''' | + | '''Use of this site is subject to you reading and agreeing with the terms set forth in the [[HemOnc.org_-_A_Hematology_Oncology_Wiki:General_disclaimer|disclaimer]]. If this is your first time visiting, we suggest you read the [[tutorial]].''' |
− | Is there a regimen missing from this list? | + | Is there a regimen missing from this list? Would you like to share a different dosage/schedule or an additional reference for a regimen? Have you noticed an error? Do you have an idea that will help the site grow to better meet your needs and the needs of many others? You are [[How_to_contribute|invited to contribute to the site]]. |
{| class="wikitable" style="text-align:center; width:50%;" | {| class="wikitable" style="text-align:center; width:50%;" | ||
!colspan="2" align="center" style="color:white; font-size:125%; background-color:#de2d26"|'''Section editor''' | !colspan="2" align="center" style="color:white; font-size:125%; background-color:#de2d26"|'''Section editor''' | ||
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− | |<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] | + | |<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Regimen |limit=10000|format=sum}} regimens on this page</b></font></div> |
− | <div style="background-color: #deebf6; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] | + | <div style="background-color: #deebf6; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Variant |limit=10000|format=sum}} variants on this page</b></font></div> |
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{{TOC limit|limit=3}} | {{TOC limit|limit=3}} | ||
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==ELN== | ==ELN== | ||
*'''2011:''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4979120/ Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet] | *'''2011:''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4979120/ Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet] | ||
+ | |||
+ | =="How I Treat"== | ||
+ | *'''2014:''' Vannucchi AM. How I treat polycythemia vera. Blood. 2014 Nov 20;124(22):3212-20. Epub 2014 Oct 2. [http://www.bloodjournal.org/content/124/22/3212.long link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/25278584 PubMed] | ||
+ | |||
+ | =First-line therapy= | ||
+ | ==Hydroxyurea monotherapy {{#subobject:511984|Regimen=1}}== | ||
+ | |||
+ | ===Regimen {{#subobject:84f020|Variant=1}}=== | ||
+ | {| class="wikitable" style="width: 100%; text-align:center;" | ||
+ | !Study | ||
+ | ![[Levels_of_Evidence#Evidence|Evidence]] | ||
+ | !Comparator | ||
+ | ![[Levels_of_Evidence#Efficacy|Efficacy]] | ||
+ | |- | ||
+ | |[http://www.bloodjournal.org/content/90/9/3370.long Najean et al. 1997 (FPSG)] | ||
+ | |style="background-color:#1a9851"|Phase III | ||
+ | |Pipobroman | ||
+ | |style="background-color:#1a9850"|Superior OS (*) | ||
+ | |- | ||
+ | |} | ||
+ | ''The following is the approach recommended by Vannucchi in How I Treat (2014). Efficacy is based on the 2011 update.'' | ||
+ | ====Chemotherapy==== | ||
+ | *[[Hydroxyurea (Hydrea)]] 500 mg PO BID, with titration based on the target hematocrit and hematologic toxicity | ||
+ | |||
+ | '''Continued until treatment failure or intolerance''' | ||
+ | |||
+ | ===References=== | ||
+ | # Najean Y, Rain JD. Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood. 1997 Nov 1;90(9):3370-7. [http://www.bloodjournal.org/content/90/9/3370.long link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/9345019 PubMed] | ||
+ | ## '''Update:''' Kiladjian JJ, Chevret S, Dosquet C, Chomienne C, Rain JD. Treatment of polycythemia vera with hydroxyurea and pipobroman: final results of a randomized trial initiated in 1980. J Clin Oncol. 2011 Oct 10;29(29):3907-13. Epub 2011 Sep 12. [http://ascopubs.org/doi/full/10.1200/JCO.2011.36.0792 link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/21911721 PubMed] | ||
=Relapsed, refractory, or intolerant= | =Relapsed, refractory, or intolerant= | ||
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|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | ||
|style="background-color:#1a9851"|Phase III | |style="background-color:#1a9851"|Phase III | ||
− | |[[ | + | |[[#Ruxolitinib_monotherapy|Ruxolitinib]] |
|style="background-color:#d73027"|Inferior disease control | |style="background-color:#d73027"|Inferior disease control | ||
|- | |- | ||
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|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | ||
|style="background-color:#1a9851"|Phase III | |style="background-color:#1a9851"|Phase III | ||
− | |[[ | + | |[[#Ruxolitinib_monotherapy|Ruxolitinib]] |
|style="background-color:#d73027"|Inferior disease control | |style="background-color:#d73027"|Inferior disease control | ||
|- | |- | ||
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===References=== | ===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. [http://www.nejm.org/doi/full/10.1056/NEJMoa1409002 link to original article] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/25629741 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. [http://www.nejm.org/doi/full/10.1056/NEJMoa1409002 link to original article] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/25629741 PubMed] | ||
## '''Update:''' Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. [http://www.haematologica.org/content/101/7/821 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5004461/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/27102499 PubMed] | ## '''Update:''' Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. [http://www.haematologica.org/content/101/7/821 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5004461/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/27102499 PubMed] | ||
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|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | ||
|style="background-color:#1a9851"|Phase III | |style="background-color:#1a9851"|Phase III | ||
− | |[[ | + | |[[#Ruxolitinib_monotherapy|Ruxolitinib]] |
|style="background-color:#d73027"|Inferior disease control | |style="background-color:#d73027"|Inferior disease control | ||
|- | |- | ||
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|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | ||
|style="background-color:#1a9851"|Phase III | |style="background-color:#1a9851"|Phase III | ||
− | |[[ | + | |[[#Ruxolitinib_monotherapy|Ruxolitinib]] |
|style="background-color:#d73027"|Inferior disease control | |style="background-color:#d73027"|Inferior disease control | ||
|- | |- | ||
Line 166: | Line 194: | ||
|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | ||
|style="background-color:#1a9851"|Phase III | |style="background-color:#1a9851"|Phase III | ||
− | |[[ | + | |[[#Ruxolitinib_monotherapy|Ruxolitinib]] |
|style="background-color:#d73027"|Inferior disease control | |style="background-color:#d73027"|Inferior disease control | ||
|- | |- | ||
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===References=== | ===References=== | ||
− | # '''Review:''' Passamonti F, Lazzarino M. Treatment of polycythemia vera and essential thrombocythemia: the role of pipobroman. Leuk Lymphoma. 2003 Sep;44(9):1483-8. [http://www.tandfonline.com/doi/abs/10.3109/10428190309178768 link to original article] | + | # '''Review:''' Passamonti F, Lazzarino M. Treatment of polycythemia vera and essential thrombocythemia: the role of pipobroman. Leuk Lymphoma. 2003 Sep;44(9):1483-8. [http://www.tandfonline.com/doi/abs/10.3109/10428190309178768 link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/14565648 PubMed] |
− | [https://www.ncbi.nlm.nih.gov/pubmed/14565648 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. [http://www.nejm.org/doi/full/10.1056/NEJMoa1409002 link to original article] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/25629741 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. [http://www.nejm.org/doi/full/10.1056/NEJMoa1409002 link to original article] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/25629741 PubMed] | ||
## '''Update:''' Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. [http://www.haematologica.org/content/101/7/821 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5004461/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/27102499 PubMed] | ## '''Update:''' Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. [http://www.haematologica.org/content/101/7/821 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5004461/ link to PMC article] [https://www.ncbi.nlm.nih.gov/pubmed/27102499 PubMed] | ||
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|- | |- | ||
|} | |} | ||
− | ''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''' and '''RESPONSE-2''' was [[ | + | ''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''' and '''RESPONSE-2''' was [[#Hydroxyurea_monotherapy_2|hydroxyurea]].'' |
====Chemotherapy==== | ====Chemotherapy==== | ||
*[[Ruxolitinib (Jakafi)]] 10 mg PO twice per day | *[[Ruxolitinib (Jakafi)]] 10 mg PO twice per day | ||
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|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820/ Vannucchi et al. 2015 (RESPONSE)] | ||
|style="background-color:#1a9851"|Phase III | |style="background-color:#1a9851"|Phase III | ||
− | |[[ | + | |[[#Ruxolitinib_monotherapy|Ruxolitinib]] |
|style="background-color:#d73027"|Inferior disease control | |style="background-color:#d73027"|Inferior disease control | ||
|- | |- |
Revision as of 23:50, 4 February 2018
Use of this site is subject to you reading and agreeing with the terms set forth in the disclaimer. If this is your first time visiting, we suggest you read the tutorial.
Is there a regimen missing from this list? Would you like to share a different dosage/schedule or an additional reference for a regimen? Have you noticed an error? Do you have an idea that will help the site grow to better meet your needs and the needs of many others? You are invited to contribute to the site.
Section editor | |
---|---|
Sanjay R. Mohan, MD, MSCI Nashville, TN |
10 regimens on this page
11 variants on this page
|
Guidelines
ELN
"How I Treat"
- 2014: Vannucchi AM. How I treat polycythemia vera. Blood. 2014 Nov 20;124(22):3212-20. Epub 2014 Oct 2. link to original article PubMed
First-line therapy
Hydroxyurea monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Najean et al. 1997 (FPSG) | Phase III | Pipobroman | Superior OS (*) |
The following is the approach recommended by Vannucchi in How I Treat (2014). Efficacy is based on the 2011 update.
Chemotherapy
- Hydroxyurea (Hydrea) 500 mg PO BID, with titration based on the target hematocrit and hematologic toxicity
Continued until treatment failure or intolerance
References
- Najean Y, Rain JD. Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood. 1997 Nov 1;90(9):3370-7. link to original article PubMed
- Update: Kiladjian JJ, Chevret S, Dosquet C, Chomienne C, Rain JD. Treatment of polycythemia vera with hydroxyurea and pipobroman: final results of a randomized trial initiated in 1980. J Clin Oncol. 2011 Oct 10;29(29):3907-13. Epub 2011 Sep 12. link to original article PubMed
Relapsed, refractory, or intolerant
Anagrelide monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Vannucchi et al. 2015 (RESPONSE) | Phase III | Ruxolitinib | Inferior disease control |
To be completed
Therapy
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 link to PMC article PubMed
- Update: Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. link to original article link to PMC article PubMed
Hydroxyurea monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Vannucchi et al. 2015 (RESPONSE) | Phase III | Ruxolitinib | Inferior disease control |
The following is the approach recommended by Vannucchi in How I Treat (2014).
Chemotherapy
- Hydroxyurea (Hydrea) 500 mg PO BID, with titration based on the target hematocrit and hematologic toxicity
Continued until treatment failure or intolerance
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 link to PMC article PubMed
- Update: Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. link to original article link to PMC article PubMed
Interferon monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Vannucchi et al. 2015 (RESPONSE) | Phase III | Ruxolitinib | Inferior disease control |
To be completed; RESPONSE does not specify what type of interferon was used in this arm.
Immunotherapy
- Interferon
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 link to PMC article PubMed
- Update: Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. link to original article link to PMC article PubMed
Lenalidomide monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Vannucchi et al. 2015 (RESPONSE) | Phase III | Ruxolitinib | Inferior disease control |
To be completed
Chemotherapy
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 link to PMC article PubMed
- Update: Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. link to original article link to PMC article PubMed
Peginterferon alfa-2a monotherapy
back to top |
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.
Immunotherapy
- Peginterferon alfa-2a (Pegasys) 90 mcg SC once per week, titrated up every two weeks in the absence of toxicity to 135 mcg SC once per week
- In the absence of hematologic response, dose increased to 180 mcg 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 link to PMC article 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 link to PMC article PubMed
- Update: Masarova L, Patel KP, Newberry KJ, Cortes J, Borthakur G, Konopleva M, Estrov Z, Kantarjian H, Verstovsek S. Pegylated interferon alfa-2a in patients with essential thrombocythaemia or polycythaemia vera: a post-hoc, median 83 month follow-up of an open-label, phase 2 trial. Lancet Haematol. 2017 Apr;4(4):e165-e175. Epub 2017 Mar 10. link to original article link to PMC article PubMed
Pipobroman monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Vannucchi et al. 2015 (RESPONSE) | Phase III | Ruxolitinib | Inferior disease control |
To be completed for historical reference
Chemotherapy
References
- Review: Passamonti F, Lazzarino M. Treatment of polycythemia vera and essential thrombocythemia: the role of pipobroman. Leuk Lymphoma. 2003 Sep;44(9):1483-8. 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 link to PMC article PubMed
- Update: Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. link to original article link to PMC article PubMed
Ruxolitinib monotherapy
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Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Verstovsek et al. 2014 | Phase II | ||
Vannucchi et al. 2015 (RESPONSE) | Phase III | Standard therapy | Superior disease control |
Passamonti et al. 2016 (RESPONSE-2) | Phase III | Standard therapy | Superior 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 and RESPONSE-2 was hydroxyurea.
Chemotherapy
- 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 link to PMC article 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 link to PMC article PubMed
- Update: Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. link to original article link to PMC article PubMed
- Passamonti F, Griesshammer M, Palandri F, Egyed M, Benevolo G, Devos T, Callum J, Vannucchi AM, Sivgin S, Bensasson C, Khan M, Mounedji N, Saydam G. Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): a randomised, open-label, phase 3b study. Lancet Oncol. 2017 Jan;18(1):88-99. Epub 2016 Dec 2. link to original article contains protocol PubMed
Thalidomide monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Vannucchi et al. 2015 (RESPONSE) | Phase III | Ruxolitinib | Inferior disease control |
To be completed
Chemotherapy
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 link to PMC article PubMed
- Update: Verstovsek S, Vannucchi AM, Griesshammer M, Masszi T, Durrant S, Passamonti F, Harrison CN, Pane F, Zachee P, Kirito K, Besses C, Hino M, Moiraghi B, Miller CB, Cazzola M, Rosti V, Blau I, Mesa R, Jones MM, Zhen H, Li J, Francillard N, Habr D, Kiladjian JJ. Ruxolitinib versus best available therapy in patients with Polycythemia Vera: 80 Week follow up from the RESPONSE trial. Haematologica. 2016 Jul;101(7):821-9. Epub 2016 Apr 21. link to original article link to PMC article PubMed