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Hypereosinophilic syndrome

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For placebo or observational studies in this condition, please visit this page.

Section editor
Sanjay R. Mohan, MD, MSCI
Vanderbilt University
Nashville, TN, USA
Last updated on 2024-07-23:
9 regimens on this page
9 variants on this page


Guidelines

Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.

NCCN


All lines of therapy

Alemtuzumab monotherapy

Regimen

Study Evidence
Wagner et al. 2009 Case report

Note: treatment is to be given bi-weekly, exact days not specified; below is an example. Duration also not specified.

Targeted therapy

7-day cycles

References

  1. Case report: Wagner LA, Speckart S, Cutter B, Gleich GJ. Treatment of FIP1L1/PDGFRA-negative hypereosinophilic syndrome with alemtuzumab, an anti-CD52 antibody. J Allergy Clin Immunol. 2009 Jun;123(6):1407-8. Epub 2009 Apr 1. link to original article dosing details in abstract have been reviewed by our editors PubMed
  2. Retrospective: Strati P, Cortes J, Faderl S, Kantarjian H, Verstovsek S. Long-term follow-up of patients with hypereosinophilic syndrome treated with Alemtuzumab, an anti-CD52 antibody. Clin Lymphoma Myeloma Leuk. 2013 Jun;13(3):287-91. Epub 2012 Nov 1. link to original article link to PMC article PubMed


Benralizumab monotherapy

Regimen

Study Dates of enrollment Evidence Comparator Comparative Efficacy
Kuang et al. 2019 (NIAID 14-I-0081) 2014-2017 Randomized Phase 2, fewer than 20 pts (E-esc) Placebo Seems to have superior primary endpoint

Immunosuppressive therapy

28-day cycle for 6 or more cycles

References

  1. NIAID 14-I-0081: Kuang FL, Legrand F, Makiya M, Ware J, Wetzler L, Brown T, Magee T, Piligian B, Yoon P, Ellis JH, Sun X, Panch SR, Powers A, Alao H, Kumar S, Quezado M, Yan L, Lee N, Kolbeck R, Newbold P, Goldman M, Fay MP, Khoury P, Maric I, Klion AD. Benralizumab for PDGFRA-negative hypereosinophilic syndrome. N Engl J Med. 2019 Apr 4;380(14):1336-1346. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article PubMed NCT00001406


Cladribine & Cytarabine

Regimen

Study Dates of enrollment Evidence
Jabbour et al. 2005 1999-04 to 2001-04 Phase 2, fewer than 20 pts

Note: the timing of drug administration is in hours in the manuscript, starting at t = 0 hours.

Chemotherapy

21-day cycle, repeated once if PR/CR; see text for definition of PR

References

  1. Jabbour E, Verstovsek S, Giles F, Gandhi V, Cortes J, O'Brien S, Plunkett W, Garcia-Manero G, Jackson CE, Kantarjian H, Andreeff M. 2-Chlorodeoxyadenosine and cytarabine combination therapy for idiopathic hypereosinophilic syndrome. Cancer. 2005 Aug 1;104(3):541-6. link to original article dosing details in manuscript have been reviewed by our editors PubMed


Hydroxyurea monotherapy

Regimen

Study Dates of enrollment Evidence
Parrillo et al. 1978 1944-1968 Non-randomized, fewer than 20 pts

Chemotherapy

Continued indefinitely

References

  1. Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med. 1978 Aug;89(2):167-72. link to original article dosing details in manuscript have been reviewed by our editors PubMed


Imatinib monotherapy

Regimen

FDA-recommended dose
Study Dates of enrollment Evidence
Gleich et al. 2002 Not reported Case series
Apperley et al. 2002 (STIB2225) Not reported Phase 2, fewer than 20 pts
Pardanani et al. 2002 Not reported Phase 2, fewer than 20 pts
Cortes et al. 2003 2001-2002 Phase 2, fewer than 20 pts
Cools et al. 2003 2001-2002 Non-randomized, fewer than 20 pts
Baccarani et al. 2007 (HES0203) 2001-2007 Phase 2
Klion et al. 2003 2003 Non-randomized, fewer than 20 pts
Helbig et al. 2008 Not reported Non-randomized, fewer than 20 pts
Metzgeroth et al. 2008 Not reported Phase 2

Note: Dosing is as described in the Metzgeroth et al. 2008 paper.

Targeted therapy

  • Imatinib (Gleevec) by the following mutation-specific criteria:
    • FIP1L1-PDGFRA fusion positive: 100 mg PO once per day
    • PDGFRB fusion positive or without known molecular aberration: 400 mg PO once per day

28-day cycle for 13 or more cycles

Subsequent treatment

  • Helbig et al. 2008: Patients proceeded to imatinib maintenance after 3 to 6 months of therapy which consisted of weekly dosing; dose not specified in the manuscript

References

  1. Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH. Treatment of hypereosinophilic syndrome with imatinib mesilate. Lancet. 2002 May 4;359(9317):1577-8. link to original article PubMed
  2. STIB2225: Apperley JF, Gardembas M, Melo JV, Russell-Jones R, Bain BJ, Baxter EJ, Chase A, Chessells JM, Colombat M, Dearden CE, Dimitrijevic S, Mahon FX, Marin D, Nikolova Z, Olavarria E, Silberman S, Schultheis B, Cross NC, Goldman JM. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med. 2002 Aug 15;347(7):481-7. link to original article dosing details in abstract have been reviewed by our editors PubMed
    1. Pooled update: Cheah CY, Burbury K, Apperley JF, Huguet F, Pitini V, Gardembas M, Ross DM, Forrest D, Genet P, Rousselot P, Patton N, Smith G, Dunbar CE, Ito S, Aguiar RC, Odenike O, Gimelfarb A, Cross NC, Seymour JF. Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib. Blood. 2014 Jun 5;123(23):3574-7. Epub 2014 Mar 31. link to original article link to PMC article PubMed
  3. Pardanani A, Reeder T, Porrata LF, Li CY, Tazelaar HD, Baxter EJ, Witzig TE, Cross NC, Tefferi A. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood. 2003 May 1;101(9):3391-7. Epub 2002 Dec 27. link to original article dosing details in abstract have been reviewed by our editors PubMed
  4. Cortes J, Ault P, Koller C, Thomas D, Ferrajoli A, Wierda W, Rios MB, Letvak L, Kaled ES, Kantarjian H. Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood. 2003 Jun 15;101(12):4714-6. Epub 2003 Feb 20. link to original article dosing details in manuscript have been reviewed by our editors PubMed
  5. Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, Kutok J, Clark J, Galinsky I, Griffin JD, Cross NC, Tefferi A, Malone J, Alam R, Schrier SL, Schmid J, Rose M, Vandenberghe P, Verhoef G, Boogaerts M, Wlodarska I, Kantarjian H, Marynen P, Coutre SE, Stone R, Gilliland DG. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003 Mar 27;348(13):1201-14. link to original article dosing details in abstract have been reviewed by our editors PubMed
  6. Klion AD, Robyn J, Akin C, Noel P, Brown M, Law M, Metcalfe DD, Dunbar C, Nutman TB. Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome. Blood. 2004 Jan 15;103(2):473-8. Epub 2003 Sep 22. link to original article dosing details in abstract have been reviewed by our editors PubMed
  7. HES0203: Baccarani M, Cilloni D, Rondoni M, Ottaviani E, Messa F, Merante S, Tiribelli M, Buccisano F, Testoni N, Gottardi E, de Vivo A, Giugliano E, Iacobucci I, Paolini S, Soverini S, Rosti G, Rancati F, Astolfi C, Pane F, Saglio G, Martinelli G. The efficacy of imatinib mesylate in patients with FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome: results of a multicenter prospective study. Haematologica. 2007 Sep;92(9):1173-9. Epub 2007 Aug 1. link to original article dosing details in abstract have been reviewed by our editors PubMed NCT00276926
  8. Helbig G, Stella-Hołowiecka B, Majewski M, Całbecka M, Gajkowska J, Klimkiewicz R, Moskwa A, Grzegorczyk J, Lewandowska M, Hołowiecki J. A single weekly dose of imatinib is sufficient to induce and maintain remission of chronic eosinophilic leukaemia in FIP1L1-PDGFRA-expressing patients. Br J Haematol. 2008 Apr;141(2):200-4. Epub 2008 Feb 26. link to original article dosing details in abstract have been reviewed by our editors PubMed
  9. Metzgeroth G, Walz C, Erben P, Popp H, Schmitt-Graeff A, Haferlach C, Fabarius A, Schnittger S, Grimwade D, Cross NC, Hehlmann R, Hochhaus A, Reiter A. Safety and efficacy of imatinib in chronic eosinophilic leukaemia and hypereosinophilic syndrome: a phase-II study. Br J Haematol. 2008 Dec;143(5):707-15. Epub 2008 Oct 17. link to original article dosing details in manuscript have been reviewed by our editors PubMed


Mepolizumab monotherapy

Regimen

Study Dates of enrollment Evidence Comparator Comparative Efficacy
Rothenberg et al. 2008 (GSK 100185) 2004-2006 Phase 3 (E-esc) Placebo Superior primary endpoint

Immunosuppressive therapy

21-day cycle for 8 cycles

References

  1. GSK 100185: Rothenberg ME, Klion AD, Roufosse FE, Kahn JE, Weller PF, Simon HU, Schwartz LB, Rosenwasser LJ, Ring J, Griffin EF, Haig AE, Frewer PI, Parkin JM, Gleich GJ; Mepolizumab HES Study Group. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. 2008 Mar 20;358(12):1215-28. Epub 2008 Mar 16. Erratum in: N Engl J Med. 2008 Jun 5;358(23): 2530. link to original article dosing details in manuscript have been reviewed by our editors PubMed NCT00086658
    1. Update: Roufosse FE, Kahn JE, Gleich GJ, Schwartz LB, Singh AD, Rosenwasser LJ, Denburg JA, Ring J, Rothenberg ME, Sheikh J, Haig AE, Mallett SA, Templeton DN, Ortega HG, Klion AD. Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol. 2013 Feb;131(2):461-7.e1-5. Epub 2012 Oct 4. link to original article link to PMC article PubMed


Nilotinib monotherapy

Regimen

Study Dates of enrollment Evidence
Kantarjian et al. 2006 (A2101) 2004-2005 Phase 1/2, fewer than 20 pts of this subtype

Targeted therapy

28-day cycles

References

  1. A2101: Kantarjian H, Giles F, Wunderle L, Bhalla K, O'Brien S, Wassmann B, Tanaka C, Manley P, Rae P, Mietlowski W, Bochinski K, Hochhaus A, Griffin JD, Hoelzer D, Albitar M, Dugan M, Cortes J, Alland L, Ottmann OG. Nilotinib in imatinib-resistant CML and Philadelphia chromosome-positive ALL. N Engl J Med. 2006 Jun 15;354(24):2542-51. link to original article PubMed NCT00109707
    1. Subgroup analysis: Hochhaus A, le Coutre PD, Kantarjian HM, Baccarani M, Erben P, Reiter A, McCulloch T, Fan X, Novick S, Giles FJ. Effect of the tyrosine kinase inhibitor nilotinib in patients with hypereosinophilic syndrome/chronic eosinophilic leukemia: analysis of the phase 2, open-label, single-arm A2101 study. J Cancer Res Clin Oncol. 2013 Dec;139(12):1985-93. Epub 2013 Sep 22. link to original article link to PMC article PubMed


Peginterferon alfa-2a monotherapy

Regimen

Study Evidence
Butterfield et al. 2011 Case series

Note: The authors describe using peginterferon alfa-2b initially but switched to peginterferon alfa-2a when the first was "no longer available. Dosing details are not available in the abstract."

Immunotherapy

References

  1. Case series: Butterfield JH, Weiler CR. Use of pegylated interferon in hypereosinophilic syndrome. Leuk Res. 2012 Feb;36(2):192-7. Epub 2011 Nov 26. link to original article PubMed


Ruxolitinib monotherapy

Regimen

Study Evidence
Rumi et al. 2013 Case report

Targeted therapy

Duration not specified

References

  1. Case report: Rumi E, Milosevic JD, Casetti I, Dambruoso I, Pietra D, Boveri E, Boni M, Bernasconi P, Passamonti F, Kralovics R, Cazzola M. Efficacy of ruxolitinib in chronic eosinophilic leukemia associated with a PCM1-JAK2 fusion gene. J Clin Oncol. 2013 Jun 10;31(17):e269-71. Epub 2013 Apr 29. link to original article dosing details in abstract have been reviewed by our editors PubMed


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