Essential thrombocythemia

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8 regimens on this page
9 variants on this page


Untreated

Aspirin & Anagrelide

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Regimen #1

Study Evidence Comparator
Harrison et al. 2005 (MRC PT-1) Phase III Aspirin & Hydroxyurea
  • Anagrelide (Agrylin) 0.5 mg PO twice daily ("Doses were subsequently adjusted to maintain the platelet count at less than 400,000 per cubic millimeter.")
  • Aspirin 75 mg (100 mg in Australia) PO once daily

Regimen #2

Study Evidence Comparator
Gisslinger et al. 2013 (ANAHYDRET) Phase III Hydroxyurea +/- Aspirin
  • Anagrelide (Agrylin) 0.5 mg PO twice daily ("increased until maintenance of the platelet count at normal (=450 x 109/L) or close to normal levels (>450 x 109/L to 600 x 109/L)")
  • Neither aspirin nor Clopidogrel (Plavix) were required in the study, but patients who were already taking either drug for at least two weeks were allowed to continue taking it during the study per investigator discretion

References

  1. Harrison CN, Campbell PJ, Buck G, Wheatley K, East CL, Bareford D, Wilkins BS, van der Walt JD, Reilly JT, Grigg AP, Revell P, Woodcock BE, Green AR; United Kingdom Medical Research Council Primary Thrombocythemia 1 Study. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005 Jul 7;353(1):33-45. link to original article contains verified protocol PubMed content property of HemOnc.org
  2. Gisslinger H, Gotic M, Holowiecki J, Penka M, Thiele J, Kvasnicka HM, Kralovics R, Petrides PE; for all members of the ANAHYDRET Study Group. Anagrelide compared with hydroxyurea in WHO-classified essential thrombocythemia: the ANAHYDRET Study, a randomized controlled trial. Blood. 2013 Mar 7;121(10):1720-8. doi: 10.1182/blood-2012-07-443770. Epub 2013 Jan 11. link to original article contains verified protocol PubMed

Aspirin & Hydroxyurea

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Regimen #1

Study Evidence Comparator
Harrison et al. 2005 (MRC PT-1) Phase III Aspirin & Anagrelide
  • Hydroxyurea (Hydrea) 500-1000 mg PO once daily ("Doses were subsequently adjusted to maintain the platelet count at less than 400,000 per cubic millimeter.")
  • Aspirin 75 mg (100 mg in Australia) PO once daily

Regimen #2

Study Evidence Comparator
Gisslinger et al. 2013 (ANAHYDRET) Phase III Anagrelide +/- Aspirin
  • Hydroxyurea (Hydrea) 1500 mg PO once daily ("increased until maintenance of the platelet count at normal (=450 x 109/L) or close to normal levels (>450 x 109/L to 600 x 109/L)")
  • Neither aspirin nor Clopidogrel (Plavix) were required in the study, but patients who were already taking either drug for at least two weeks were allowed to continue taking it during the study per investigator discretion

References

  1. Harrison CN, Campbell PJ, Buck G, Wheatley K, East CL, Bareford D, Wilkins BS, van der Walt JD, Reilly JT, Grigg AP, Revell P, Woodcock BE, Green AR; United Kingdom Medical Research Council Primary Thrombocythemia 1 Study. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005 Jul 7;353(1):33-45. link to original article contains verified protocol PubMed
  2. Gisslinger H, Gotic M, Holowiecki J, Penka M, Thiele J, Kvasnicka HM, Kralovics R, Petrides PE; for all members of the ANAHYDRET Study Group. Anagrelide compared with hydroxyurea in WHO-classified essential thrombocythemia: the ANAHYDRET Study, a randomized controlled trial. Blood. 2013 Mar 7;121(10):1720-8. Epub 2013 Jan 11. link to original article contains verified protocol PubMed

Hydroxyurea (Hydrea)

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Regimen

Study Evidence Comparator
Cortelazzo et al. 1995 Phase III Observation
  • Hydroxyurea (Hydrea) 15 mg/kg/day PO
    • Doses were subsequently adjusted to maintain the platelet count at less than 600,000 per cubic millimeter without lowering the white-cell count below 4000 per cubic millimeter

References

  1. Cortelazzo S, Finazzi G, Ruggeri M, Vestri O, Galli M, Rodeghiero F, Barbui T. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995 Apr 27;332(17):1132-6. link to original article contains verified protocol PubMed

Observation

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Regimen

Study Evidence Comparator
Cortelazzo et al. 1995 Phase III Hydroxyurea

No active treatment; used as a comparator arm.

References

  1. Cortelazzo S, Finazzi G, Ruggeri M, Vestri O, Galli M, Rodeghiero F, Barbui T. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995 Apr 27;332(17):1132-6. link to original article contains verified protocol PubMed

Peginterferon alfa-2a (Pegasys)

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Regimen

Study Evidence
Quintás-Cardama et al. 2009 Phase II

Quintás-Cardama et al. 2009 do not provide guidance on dose escalation or target dosing.

Unspecified duration

References

  1. 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

Relapsed/Refractory

Peginterferon alfa-2a (Pegasys)

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Regimen

Study Evidence
Verger et al. 2015 Observational

Verger et al. 2015 do not specify a protocol but rather point to the European LeukemiaNet guidelines from 2011. All patients in this study had CALR mutations.

References

  1. Verger E, Cassinat B, Chauveau A, Dosquet C, Giraudier S, Schlageter MH, Ianotto JC, Yassin MA, Al-Dewik N, Carillo S, Legouffe E, Ugo V, Chomienne C, Kiladjian JJ. Clinical and molecular response to interferon-α therapy in essential thrombocythemia patients with CALR mutations. Blood. 2015 Dec 10;126(24):2585-91. Epub 2015 Oct 20. link to original article does not contain protocol PubMed