Polycythemia vera medical therapy: Difference between revisions

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{{CMG}} {{AE}} {{MJK}}
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==Overview==
==Overview==
The mainstay of therapy for polycythemia vera is [[phlebotomy]], [[hydroxyurea]] (alone or with phlebotomy), interferon-alpha and pegylated interferon-alpha, chlorambucil, and low-dose aspirin (≤100 mg) daily.<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_5</ref>
The mainstay of therapy for polycythemia vera is [[phlebotomy]], [[hydroxyurea]] (alone or with phlebotomy), [[interferon-alpha]] and pegylated interferon-alpha, [[chlorambucil]], and low-dose [[aspirin]].<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_5</ref>
==Medical Therapy==
==Medical Therapy==
treatment focuses on treating symptoms and reducing thrombotic complications reducing the erythrocyte levels.
Medical therapy for polycythemia vera include:<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_5</ref><ref name="pmid3704665">{{cite journal| author=Berk PD, Goldberg JD, Donovan PB, Fruchtman SM, Berlin NI, Wasserman LR| title=Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. | journal=Semin Hematol | year= 1986 | volume= 23 | issue= 2 | pages= 132-43 | pmid=3704665 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3704665  }} </ref><ref name="pmid9209196">{{cite journal| author=Lamy T, Devillers A, Bernard M, Moisan A, Grulois I, Drenou B et al.| title=Inapparent polycythemia vera: an unrecognized diagnosis. | journal=Am J Med | year= 1997 | volume= 102 | issue= 1 | pages= 14-20 | pmid=9209196 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9209196  }} </ref><ref name="pmid3749925">{{cite journal| author=Kaplan ME, Mack K, Goldberg JD, Donovan PB, Berk PD, Wasserman LR| title=Long-term management of polycythemia vera with hydroxyurea: a progress report. | journal=Semin Hematol | year= 1986 | volume= 23 | issue= 3 | pages= 167-71 | pmid=3749925 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3749925  }} </ref><ref name="pmid10803930">{{cite journal| author=Lengfelder E, Berger U, Hehlmann R| title=Interferon alpha in the treatment of polycythemia vera. | journal=Ann Hematol | year= 2000 | volume= 79 | issue= 3 | pages= 103-9 | pmid=10803930 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10803930  }} </ref><ref name="pmid16804923">{{cite journal| author=Silver RT| title=Long-term effects of the treatment of polycythemia vera with recombinant interferon-alpha. | journal=Cancer | year= 2006 | volume= 107 | issue= 3 | pages= 451-8 | pmid=16804923 | doi=10.1002/cncr.22026 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16804923  }} </ref><ref name="pmid25069759">{{cite journal| author=Huang BT, Zeng QC, Zhao WH, Li BS, Chen RL| title=Interferon α-2b gains high sustained response therapy for advanced essential thrombocythemia and polycythemia vera with JAK2V617F positive mutation. | journal=Leuk Res | year= 2014 | volume= 38 | issue= 10 | pages= 1177-83 | pmid=25069759 | doi=10.1016/j.leukres.2014.06.019 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25069759 }} </ref><ref name="pmid19826111">{{cite journal| author=Quintás-Cardama A, Kantarjian H, Manshouri T, Luthra R, Estrov Z, Pierce S et al.| title=Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera. | journal=J Clin Oncol | year= 2009 | volume= 27 | issue= 32 | pages= 5418-24 | pmid=19826111 | doi=10.1200/JCO.2009.23.6075 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19826111  }} </ref><ref name="pmid23782935">{{cite journal| author=Quintás-Cardama A, Abdel-Wahab O, Manshouri T, Kilpivaara O, Cortes J, Roupie AL et al.| title=Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a. | journal=Blood | year= 2013 | volume= 122 | issue= 6 | pages= 893-901 | pmid=23782935 | doi=10.1182/blood-2012-07-442012 | pmc=PMC3739035 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23782935  }} </ref><ref name="pmid17264301">{{cite journal| author=Finazzi G, Barbui T| title=How I treat patients with polycythemia vera. | journal=Blood | year= 2007 | volume= 109 | issue= 12 | pages= 5104-11 | pmid=17264301 | doi=10.1182/blood-2006-12-038968 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17264301  }} </ref><ref name="pmid23633335">{{cite journal| author=Squizzato A, Romualdi E, Passamonti F, Middeldorp S| title=Antiplatelet drugs for polycythaemia vera and essential thrombocythaemia. | journal=Cochrane Database Syst Rev | year= 2013 | volume= 4 | issue=  | pages= CD006503 | pmid=23633335 | doi=10.1002/14651858.CD006503.pub3 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23633335  }} </ref>
 
*[[Phlebotomy]]
[[Bloodletting]] or phlebotomy is one form of treatment, which often may be combined with other therapies. The removal of blood from the body reduces the blood volume and brings down the hematocrit levels; in patients with polycythemia vera, this reduces the risk of blood clots. Phlebotomy is typically performed in people with polycythemia vera to bring their [[hematocrit]] (red blood cell percentage) down below 45 for men or 42 for women.<ref>{{cite journal |author=Streiff MB, Smith B, Spivak JL |title=The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Study Group: a survey of American Society of Hematology members' practice patterns |journal=Blood |volume=99 |issue=4 |pages=1144-9 |year=2002 |pmid=11830459}}</ref>
*[[Hydroxyurea]]
 
*[[Interferon-alpha]]
Low dose [[aspirin]] is often prescribed.  Research has shown that aspirin reduces the risk for various thrombotic complications.
*Pegylated interferon-alpha
 
*[[Chlorambucil]] or [[busulfan]]
[[Chemotherapy]] for polycythemia may be used sparingly, when the rate of bloodlettings required to maintain normal hematocrit is not acceptable. This is usually with a "cytoreductive agent" ([[hydroxyurea]], also known as [[hydroxycarbamide]]).
:*If interferon or hydroxyurea are not tolerated
 
:*Preferred for patients older than 70 years
The tendency to avoid chemotherapy if possible, especially in young patients, is due to research indicating increased risk of transformation to [[acute myelogenous leukemia|AML]], and while hydroxyurea is considered safer in this aspect, there is still some debate about its long-term safety.
*Low-dose [[aspirin]] (≤100 mg) daily
 
In the past, injection of radioactive isotopes was used as another means to suppress the bone marrow. Such treatment is now avoided due to a high rate of AML transformation.
 
Other therapies include [[interferon]] injections, and in cases where secondary [[thrombocytosis]] (high [[platelet]] count) is present, [[anagrelide]] may be prescribed.
 
[[Bone marrow transplant]]s are rarely undertaken in polycythemia patients - since this condition is non-fatal if treated and monitored, the benefits rarely outweigh the risks involved in such a procedure.


==References==
==References==

Revision as of 17:46, 6 November 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]

Overview

The mainstay of therapy for polycythemia vera is phlebotomy, hydroxyurea (alone or with phlebotomy), interferon-alpha and pegylated interferon-alpha, chlorambucil, and low-dose aspirin.[1]

Medical Therapy

Medical therapy for polycythemia vera include:[1][2][3][4][5][6][7][8][9][10][11]

  • If interferon or hydroxyurea are not tolerated
  • Preferred for patients older than 70 years

References

  1. ↑ 1.0 1.1 National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_5
  2. ↑ Berk PD, Goldberg JD, Donovan PB, Fruchtman SM, Berlin NI, Wasserman LR (1986). "Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols". Semin Hematol. 23 (2): 132–43. PMID 3704665.
  3. ↑ Lamy T, Devillers A, Bernard M, Moisan A, Grulois I, Drenou B; et al. (1997). "Inapparent polycythemia vera: an unrecognized diagnosis". Am J Med. 102 (1): 14–20. PMID 9209196.
  4. ↑ Kaplan ME, Mack K, Goldberg JD, Donovan PB, Berk PD, Wasserman LR (1986). "Long-term management of polycythemia vera with hydroxyurea: a progress report". Semin Hematol. 23 (3): 167–71. PMID 3749925.
  5. ↑ Lengfelder E, Berger U, Hehlmann R (2000). "Interferon alpha in the treatment of polycythemia vera". Ann Hematol. 79 (3): 103–9. PMID 10803930.
  6. ↑ Silver RT (2006). "Long-term effects of the treatment of polycythemia vera with recombinant interferon-alpha". Cancer. 107 (3): 451–8. doi:10.1002/cncr.22026. PMID 16804923.
  7. ↑ Huang BT, Zeng QC, Zhao WH, Li BS, Chen RL (2014). "Interferon α-2b gains high sustained response therapy for advanced essential thrombocythemia and polycythemia vera with JAK2V617F positive mutation". Leuk Res. 38 (10): 1177–83. doi:10.1016/j.leukres.2014.06.019. PMID 25069759.
  8. ↑ Quintás-Cardama A, Kantarjian H, Manshouri T, Luthra R, Estrov Z, Pierce S; et al. (2009). "Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera". J Clin Oncol. 27 (32): 5418–24. doi:10.1200/JCO.2009.23.6075. PMID 19826111.
  9. ↑ Quintás-Cardama A, Abdel-Wahab O, Manshouri T, Kilpivaara O, Cortes J, Roupie AL; et al. (2013). "Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a". Blood. 122 (6): 893–901. doi:10.1182/blood-2012-07-442012. PMC 3739035. PMID 23782935.
  10. ↑ Finazzi G, Barbui T (2007). "How I treat patients with polycythemia vera". Blood. 109 (12): 5104–11. doi:10.1182/blood-2006-12-038968. PMID 17264301.
  11. ↑ Squizzato A, Romualdi E, Passamonti F, Middeldorp S (2013). "Antiplatelet drugs for polycythaemia vera and essential thrombocythaemia". Cochrane Database Syst Rev. 4: CD006503. doi:10.1002/14651858.CD006503.pub3. PMID 23633335.

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