Best Practice & Research Clinical Rheumatology
Volume 22, Issue 5 , Pages 863-882 , October 2008

Hypereosinophilic syndromes

  • Loïc Guillevin, MD

      Affiliations

    • Corresponding Author InformationCorresponding author. Assistance Publique Hôpitaux de Paris, Université Paris Descartes, 27 Rue du Faubourg St. Jacques, 75679 Paris Cedex 14, France. Tel.: +33 1 58 41 13 21; Fax: +33 1 58 41 14 60.

References 

  1. Hardy W, Anderson R. The hypereosinophilic syndromes. Annals of Internal Medicine. 1968;68:1220–1229
  2. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine. 1975;54(1):1–27
  3. Hogan SP, Rosenberg HF, Moqbel R, et al. Eosinophils: biological properties and role in health and disease. Clinical and Experimental Allergy. 2008;38(5):709–750
  4. Rothenberg ME, Hogan SP. The eosinophil. Annual Review of Immunology. 2006;24:147–174
  5. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine. 1984;63(2):65–81
  6. Tsukadaira A, Okubo Y, Kitano K, et al. Eosinophil active cytokines and surface analysis of eosinophils in Churg-Strauss syndrome. Allergy and Asthma Proceedings. 1999;20(1):39–44
  7. Termeer CC, Simon JC, Schopf E. Low-dose interferon alfa-2b for the treatment of Churg-Strauss syndrome with prominent skin involvement. Archives of Dermatology. 2001;137(2):136–138
  8. Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Annals of Internal Medicine. 1998;129(5):370–374
  9. Tai PC, Holt ME, Denny P, et al. Deposition of eosinophil cationic protein in granulomas in allergic granulomatosis and vasculitis: the Churg-Strauss syndrome. British Medical Journal (Clinical Research Ed.). 1984;289(6442):400–402
  10. Peen E, Hahn P, Lauwers G, et al. Churg-Strauss syndrome: localization of eosinophil major basic protein in damaged tissues. Arthritis and Rheumatism. 2000;43(8):1897–1900
  11. Xiao H, Heeringa P, Hu P, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. The Journal of Clinical Investigation. 2002;110(7):955–963
  12. Guilpain P, Servettaz A, Goulvestre C, et al. Pathogenic effects of antimyeloperoxidase antibodies in patients with microscopic polyangiitis. Arthritis and Rheumatism. 2007;56(7):2455–2463
  13. Lane SE, Watts RA, Bentham G, et al. Are environmental factors important in primary systemic vasculitis? A case-control study. Arthritis and Rheumatism. 2003;48(3):814–823
  14. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine. 1999;78(1):26–37
  15. Wechsler ME, Finn D, Gunawardena D, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. 2000;117(3):708–713
  16. Wechsler ME, Pauwels R, Drazen JM. Leukotriene modifiers and Churg-Strauss syndrome: adverse effect or response to corticosteroid withdrawal?. Drug Safety. 1999;21(4):241–251
  17. Rothenberg ME, Klion AD, Roufosse FE, et al. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. The New England Journal of Medicine. 2008;358(12):1215–1228
  18. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994;83(10):2759–2779
  19. Ogbogu PU, Rosing DR, Horne MK. Cardiovascular manifestations of hypereosinophilic syndromes. Immunology and Allergy Clinics of North America. 2007;27(3):457–475
  20. Moore PM, Harley JB, Fauci AS. Neurologic dysfunction in the idiopathic hypereosinophilic syndrome. Annals of Internal Medicine. 1985;102(1):109–114
  21. Krahn M, Lopez de Munain A, Streichenberger N, et al. CAPN3 mutations in patients with idiopathic eosinophilic myositis. Annals of Neurology. 2006;59(6):905–911
  22. Butterfield JH, Leiferman KM, Gleich GJ. Nodules, eosinophilia, rheumatism, dermatitis and swelling (NERDS): a novel eosinophilic disorder. Clinical and Experimental Allergy. 1993;23(7):571–580
  23. Sable-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Annals of Internal Medicine. 2005;143(9):632–638
  24. Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine. 2005;84(2):115–128
  25. Klion AD, Bochner BS, Gleich GJ, et al. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. The Journal of Allergy and Clinical Immunology. 2006;117(6):1292–1302
  26. Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. The New England Journal of Medicine. 2003;348(13):1201–1214
  27. Buijs A, Bruin M. Fusion of FIP1L1 and RARA as a result of a novel t(4;17)(q12;q21) in a case of juvenile myelomonocytic leukemia. Leukemia. 2007;21(5):1104–1108
  28. Joosten PH, Toepoel M, Mariman EC, Van Zoelen EJ. Promoter haplotype combinations of the platelet-derived growth factor alpha-receptor gene predispose to human neural tube defects. Nature Genetics. 2001;27(2):215–217
  29. Buitenhuis M, Verhagen LP, Cools J, Coffer PJ. Molecular mechanisms underlying FIP1L1-PDGFRA-mediated myeloproliferation. Cancer Research. 2007;67(8):3759–3766
  30. Crescenzi B, Chase A, Starza RL, et al. FIP1L1-PDGFRA in chronic eosinophilic leukemia and BCR-ABL1 in chronic myeloid leukemia affect different leukemic cells. Leukemia. 2007;21(3):397–402
  31. Robyn J, Lemery S, McCoy JP, et al. Multilineage involvement of the fusion gene in patients with FIP1L1/PDGFRA-positive hypereosinophilic syndrome. British Journal of Haematology. 2006;132(3):286–292
  32. Yamada Y, Rothenberg ME, Lee AW, et al. The FIP1L1-PDGFRA fusion gene cooperates with IL-5 to induce murine hypereosinophilic syndrome (HES)/chronic eosinophilic leukemia (CEL)-like disease. Blood. 2006;107(10):4071–4079
  33. Rives S, Alcorta I, Toll T, et al. Idiopathic hypereosinophilic syndrome in children: report of a 7-year-old boy with FIP1L1-PDGFRA rearrangement. Journal of Pediatric Hematology/Oncology. 2005;27(12):663–665
  34. Klion AD, Noel P, Akin C, et al. Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness. Blood. 2003;101(12):4660–4666
  35. Metzgeroth G, Walz C, Score J, et al. Recurrent finding of the FIP1L1-PDGFRA fusion gene in eosinophilia-associated acute myeloid leukemia and lymphoblastic T-cell lymphoma. Leukemia. 2007;21(6):1183–1188
  36. McPherson T, Cowen EW, McBurney E, Klion AD. Platelet-derived growth factor receptor-alpha-associated hypereosinophilic syndrome and lymphomatoid papulosis. The British Journal of Dermatology. 2006;155(4):824–826
  37. Curtis CE, Grand FH, Musto P, et al. Two novel imatinib-responsive PDGFRA fusion genes in chronic eosinophilic leukaemia. British Journal of Haematology. 2007;138(1):77–81
  38. Score J, Curtis C, Waghorn K, et al. Identification of a novel imatinib responsive KIF5B-PDGFRA fusion gene following screening for PDGFRA overexpression in patients with hypereosinophilia. Leukemia. 2006;20(5):827–832
  39. Walz C, Curtis C, Schnittger S, et al. Transient response to imatinib in a chronic eosinophilic leukemia associated with ins(9;4)(q33;q12q25) and a CDK5RAP2-PDGFRA fusion gene. Genes Chromosomes Cancer. 2006;45(10):950–956
  40. Gotlib J. Molecular classification and pathogenesis of eosinophilic disorders: 2005 update. Acta Haematologica. 2005;114(1):7–25
  41. Jones AV, Kreil S, Zoi K, et al. Widespread occurrence of the JAK2 V617F mutation in chronic myeloproliferative disorders. Blood. 2005;106(6):2162–2168
  42. Helbig G, Majewski M, Wieczorkiewicz A, et al. Screening for JAK2 V617F point mutation in patients with hypereosinophilic syndrome-in response to ‘Hypereosinophilic syndrome: another face of Janus?’ by Dahabreh, et al. published in Leukemia Research. Leukemia Research, in press.
  43. Helbig G, Stella-Holowiecka B, Majewski M, et al. Interferon alpha induces a good molecular response in a patient with chronic eosinophilic leukemia (CEL) carrying the JAK2V617F point mutation. Haematologica. 2007;92(11):e118–e119
  44. Cogan E, Schandene L, Crusiaux A, et al. Brief report: clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome. The New England Journal of Medicine. 1994;330(8):535–538
  45. Simon HU, Plotz SG, Dummer R, Blaser K. Abnormal clones of T cells producing interleukin-5 in idiopathic eosinophilia. The New England Journal of Medicine. 1999;341(15):1112–1120
  46. Roufosse F, Cogan E, Goldman M. Recent advances in pathogenesis and management of hypereosinophilic syndromes. Allergy. 2004;59(7):673–689
  47. Willard-Gallo KE, Badran BM, Ravoet M, et al. Defective CD3gamma gene transcription is associated with NFATc2 overexpression in the lymphocytic variant of hypereosinophilic syndrome. Experimental Hematology. 2005;33(10):1147–1159
  48. Roufosse F, Cogan E, Goldman M. The hypereosinophilic syndrome revisited. Annual Review of Medicine. 2003;54:169–184
  49. Ravoet M, Sibille C, Roufosse F, et al. 6q- is an early and persistent chromosomal aberration in CD3-CD4+ T-cell clones associated with the lymphocytic variant of hypereosinophilic syndrome. Haematologica. 2005;90(6):753–765
  50. Galimberti S, Ciabatti E, Ottimo F, et al. Cell clonality in hypereosinophilic syndrome: what pathogenetic role?. Clinical and Experimental Rheumatology. 2007;25(1):17–22
  51. Roche-Lestienne C, Lepers S, Soenen-Cornu V, et al. Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics. Leukemia. 2005;19(5):792–798
  52. Gleich GJ, Leiferman KM, Pardanani A, et al. Treatment of hypereosinophilic syndrome with imatinib mesilate. Lancet. 2002;359(9317):1577–1578
  53. Pardanani A, Reeder T, Porrata LF, et al. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood. 2003;101(9):3391–3397
  54. Jovanovic JV, Score J, Waghorn K, et al. Low-dose imatinib mesylate leads to rapid induction of major molecular responses and achievement of complete molecular remission in FIP1L1-PDGFRA-positive chronic eosinophilic leukemia. Blood. 2007;109(11):4635–4640
  55. Baccarani M, Cilloni D, Rondoni M, et al. The efficacy of imatinib mesylate in patients with FIP1l1-PDGFRalpha-positive hypereosinophilic syndrome. Results of a multicenter prospective study. Haematologica. 2007;
  56. Helbig G, Stella-Holowiecka B, Majewski M, et al. A single weekly dose of imatinib is sufficient to induce and maintain remission of chronic eosinophilic leukaemia in FIP1L1-PDGFRA-expressing patients. British Journal of Haematology. 2008;141(2):200–204
  57. Simon D, Salemi S, Yousefi S, Simon HU. Primary resistance to imatinib in Fip1-like 1-platelet-derived growth factor receptor alpha-positive eosinophilic leukemia. The Journal of Allergy and Clinical Immunology. 2008;121(4):1054–1056
  58. von Bubnoff N, Sandherr M, Schlimok G, et al. Myeloid blast crisis evolving during imatinib treatment of an FIP1L1-PDGFR alpha-positive chronic myeloproliferative disease with prominent eosinophilia. Leukemia. 2005;19(2):286–287
  59. Cools J, Stover EH, Boulton CL, et al. PKC412 overcomes resistance to imatinib in a murine model of FIP1L1-PDGFRalpha-induced myeloproliferative disease. Cancer Cell. 2003;3(5):459–469
  60. Lierman E, Folens C, Stover EH, et al. Sorafenib is a potent inhibitor of FIP1L1-PDGFRalpha and the imatinib-resistant FIP1L1-PDGFRalpha T674I mutant. Blood. 2006;108(4):1374–1376
  61. Verstovsek S, Tefferi A, Cortes J, et al. Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis. Clinical Cancer Research. 2008;14(12):3906–3915
  62. Pitini V, Arrigo C, Azzarello D, et al. Serum concentration of cardiac Troponin T in patients with hypereosinophilic syndrome treated with imatinib is predictive of adverse outcomes. Blood. 2003;102(9):3456–3457author reply 7
  63. Rotoli B, Catalano L, Galderisi M, et al. Rapid reversion of Loeffler's endocarditis by imatinib in early stage clonal hypereosinophilic syndrome. Leukemia & Lymphoma. 2004;45(12):2503–2507
  64. Klion AD, Robyn J, Akin C, et al. Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome. Blood. 2004;103(2):473–478
  65. Klion AD, Robyn J, Maric I, et al. Relapse following discontinuation of imatinib mesylate therapy for FIP1L1/PDGFRA-positive chronic eosinophilic leukemia: implications for optimal dosing. Blood. 2007;
  66. Apperley JF, Gardembas M, Melo JV, et al. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. The New England Journal of Medicine. 2002;347(7):481–487
  67. Coutant G, Bletry O, Prin L, et al. Treatment of hypereosinophilic syndromes of myeloproliferative expression with the combination of hydroxyurea and interferon alpha. Apropos of 7 cases. Annales de médecine interne. 1993;144(4):243–250
  68. Ueno NT, Anagnostopoulos A, Rondon G, et al. Successful non-myeloablative allogeneic transplantation for treatment of idiopathic hypereosinophilic syndrome. British Journal of Haematology. 2002;119(1):131–134
  69. Leckie MJ, ten Brinke A, Khan J, et al. Effects of an interleukin-5 blocking monoclonal antibody on eosinophils, airway hyper-responsiveness, and the late asthmatic response. Lancet. 2000;356(9248):2144–2148
  70. Phipps S, Flood-Page P, Menzies-Gow A, et al. Intravenous anti-IL-5 monoclonal antibody reduces eosinophils and tenascin deposition in allergen-challenged human atopic skin. The Journal of Investigative Dermatology. 2004;122(6):1406–1412
  71. Menzies-Gow A, Flood-Page P, Sehmi R, et al. Anti-IL-5 (mepolizumab) therapy induces bone marrow eosinophil maturational arrest and decreases eosinophil progenitors in the bronchial mucosa of atopic asthmatics. The Journal of Allergy and Clinical Immunology. 2003;111(4):714–719
  72. Plotz SG, Simon HU, Darsow U, et al. Use of an anti-interleukin-5 antibody in the hypereosinophilic syndrome with eosinophilic dermatitis. The New England Journal of Medicine. 2003;349(24):2334–2339
  73. Garrett JK, Jameson SC, Thomson B, et al. Anti-interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes. The Journal of Allergy and Clinical Immunology. 2004;113(1):115–119
  74. Buttner C, Lun A, Splettstoesser T, et al. Monoclonal anti-interleukin-5 treatment suppresses eosinophil but not T-cell functions. The European Respiratory Journal. 2003;21(5):799–803
  75. Schandene L, Roufosse F, de Lavareille A, et al. Interferon alpha prevents spontaneous apoptosis of clonal Th2 cells associated with chronic hypereosinophilia. Blood. 2000;96(13):4285–4292
  76. Schandene L, Del Prete GF, Cogan E, et al. Recombinant interferon-alpha selectively inhibits the production of interleukin-5 by human CD4+ T cells. The Journal of Clinical Investigation. 1996;97(2):309–315
  77. Aldebert D, Lamkhioued B, Desaint C, et al. Eosinophils express a functional receptor for interferon alpha: inhibitory role of interferon alpha on the release of mediators. Blood. 1996;87(6):2354–2360
  78. Broxmeyer HE, Lu L, Platzer E, et al. Comparative analysis of the influences of human gamma, alpha and beta interferons on human multipotential (CFU-GEMM), erythroid (BFU-E) and granulocyte-macrophage (CFU-GM) progenitor cells. Journal of Immunology (Baltimore, Md. : 1950). 1983;131(3):1300–1305
  79. Pitini V, Teti D, Arrigo C, Righi M. Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome with abnormal T cells: a case report. British Journal of Haematology. 2004;127(5):477
  80. Sefcick A, Sowter D, DasGupta E, et al. Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome. British Journal of Haematology. 2004;124(4):558–559
  81. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine. 1996;75(1):17–28
  82. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. The New England Journal of Medicine. 2003;349(1):36–44
  83. Martinez V, Cohen P, Pagnoux C, et al. Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis and Rheumatism. 2008;58(1):308–317
  84. Keogh KA, Wylam ME, Stone JH, Specks U. Induction of remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis and Rheumatism. 2005;52(1):262–268
  85. Kaushik VV, Reddy HV, Bucknall RC. Successful use of rituximab in a patient with recalcitrant Churg-Strauss syndrome. Annals of the Rheumatic Diseases. 2006;65(8):1116–1117
  86. Koukoulaki M, Smith KG, Jayne DR. Rituximab in Churg-Strauss syndrome. Annals of the Rheumatic Diseases. 2006;65(4):557–559
  87. Winchester DE, Jacob A, Murphy T. Omalizumab for asthma. The New England Journal of Medicine. 2006;355(12):1281–1282

PII: S1521-6942(08)00110-1

doi: 10.1016/j.berh.2008.09.010

Best Practice & Research Clinical Rheumatology
Volume 22, Issue 5 , Pages 863-882 , October 2008