, Volume 7, Issue 5, pp 341–355 | Cite as

Hypereosinophilic Syndromes

Established and New Treatment Options
  • Joseph H. Butterfield
Disease Management


Knowledge of the cause(s) of the hypereosinophilic syndromes (HES) would greatly improve treatment options. Although it is unlikely that all patients with the HES will respond equally well to the same treatment, the use of currently available agents to lower the total eosinophil count to <109/L can be lifesaving. Until recently, treatment of HES has been modelled after that of other haematological disorders by using combinations of various cytotoxic agents. This approach has led to the development of the current standard regimens of therapy. With improved understanding of the effects of corticosteroids and interferon-α (IFNα) on eosinophilopoiesis and on the actions of eosinophil-active cytokines; a new plateau has been reached where agents are targeted specifically to defeat uncontrolled eosinophilopoiesis.

We now consider IFNα a first line agent for new HES patients as well as for controlling eosinophilia resistant to conventional therapy. IFNα as a single agent is effective for long term control of HES. Most patients respond to a dosage of 2 to 9 MU/day. Frequently, the dosage can be lowered after eosinophilia has been controlled. Sudden discontinuation of IFNα has resulted in recurrence of eosinophilia within weeks. If a suboptimal response occurs, prednisone or hydroxycarbamide (hydroxyurea) can be added. Resistance to IFNα may indicate development of a myeloproliferative or lymphoproliferative state or of eosinophilic leukaemia.

As a new option for drug-resistant HES, allogeneic bone marrow transplantation should be considered. Although the number of patients is still small, within the past several years durable responses have been reported.

Treatment of the cardiovascular sequelae of HES remains a therapeutic challenge. Improved cardiac surgery techniques have allowed many patients to benefit from ventricular decortication and valve replacement. It is important to combine control of eosinophilia with any operative intervention in HES because of the danger of recurrent disease if eosinophilia remains unchecked. Long term anticoagulation is probably indicated in patients with evident cardiac thrombus or peripheral vascular emboli; however, whether HES patients should routinely receive anticoagulants and whether continued anticoagulation should be given after eosinophilia has been controlled remain unanswered questions.


Adis International Limited Eosinophilia Allergy Clin Immunol Bullous Pemphigoid Hydroxycarbamide 
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  1. 1.
    Hardy WR, Anderson RE. The hypereosinophilic syndromes. Ann Intern Med 1968 Jun; 68 (6): 1220–9PubMedGoogle Scholar
  2. 2.
    Chusid MJ, Dale DC, West BC, et al. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975 Jan; 54 (1): 1–27Google Scholar
  3. 3.
    Dale DC, Hubert RT, Fauci A. Eosinophil kinetics in the hypereosinophilic syndrome. J Lab Clin Med 1976 Mar; 87 (3): 487–95PubMedGoogle Scholar
  4. 4.
    Fauci AS, Harley JB, Roberts WC, et al. NIH conference: the idiopathic hypereosinophilic syndrome: clinical, pathophys-iologic, and therapeutic considerations. Ann Intern Med 1982 Jul; 97 (1): 78–92PubMedGoogle Scholar
  5. 5.
    da Silva MA, Heerema N, Schwenk Jr GR, et al. Evidence for the clonal nature of hypereosinophilic syndrome. Cancer Genet Cytogenet 1988 May; 32 (1): 109–15PubMedGoogle Scholar
  6. 6.
    Luppi M, Marasca R, Morselli M, et al. Clonal nature of hypereosinophilic syndrome [letter]. Blood 1994 Jul 1; 84 (1): 349–50PubMedGoogle Scholar
  7. 7.
    Malbrain ML, Van den Bergh H, Zachee R Further evidence for the clonal nature of the idiopathic hypereosinophilic syndrome: complete haematological and cytogenetic remission induced by interferon-alpha in a case with a unique chromosomal abnormality. Br J Haematol 1996 Jan; 92 (1): 176–83PubMedGoogle Scholar
  8. 8.
    Uhrbrand H. The number of circulation eosinophils: normal figures and spontaneous variations. Acta Med Scand 1958; 160: 99–104PubMedGoogle Scholar
  9. 9.
    Booth BH. Diagnosis of immediate hypersensitivity. In: Patterson R, Zeiss CR, Grammer LC, et al. editors. Allergic diseases: diagnosis and management. 4th ed. Philadelphia: JB Lippincott Company, 1993:216Google Scholar
  10. 10.
    Rosenberg M, Patterson R, Mintzer R, et al. Clinical and immunologic criteria for the diagnosis of allergic broncho-pulmonary aspergillosis. Ann Intern Med 1977 Apr; 86 (4): 405–14PubMedGoogle Scholar
  11. 11.
    Greenberger PA. Allergic bronchopulmonary aspergillosis. J Allergy Clin Immunol 1984 Nov; 74 (5): 645–53PubMedGoogle Scholar
  12. 12.
    Crofton J, Douglas A. Respiratory diseases. 2nd ed. Oxford: Blackwell Scientific Publications, 1975: 444Google Scholar
  13. 13.
    Spry C. Eosinophilia in Addison’s disease. Yale J Biol Med 1976 Sep; 49 (4): 411–3PubMedGoogle Scholar
  14. 14.
    Weller PR Eosinophilia. In: Rich RR, Fleisher TA, Schwartz BD, et al., editors. Clinical immunology: principles and practice. Vol 1. St. Louis: Mosby-Year Book, 1996: 1022–31Google Scholar
  15. 15.
    Kan H, Ogata T, Taniyama A, et al. Extraordinarily high eosinophilia and elevated serum interleukin-5 level observed in a patient infected with Paragonimus westermani. Pediatrics 1995 Aug; 96 (2): 351–4PubMedGoogle Scholar
  16. 16.
    Kueck BD, Smith RE, Parkin J, et al Eosinophilic leukemia: a myeloproliferative disorder distinct from the hypereosinophilic syndrome. Hematol Pathol 1991; 5 (4): 195–205PubMedGoogle Scholar
  17. 17.
    Lonnqvist B, Gahrton G, Eriksson P, et al Isochromosome 17 in a patient with a myeloproliferative disorder terminating in eosinophilic leukemia. Acta Med Scand 1979; 206 (4): 321–5PubMedGoogle Scholar
  18. 18.
    Chusid MJ, Dale DC. Eosinophilic leukemia: remission with vincristine and hydroxyurea. Am J Med 1975 Aug; 59 (2): 297–300PubMedGoogle Scholar
  19. 19.
    Yates P, Potter MN Eosinophilic leukaemia with an abnormality of 5q31, the site of the IL-5 gene. Clin Lab Haematol 1991; 13 (2): 211–5PubMedGoogle Scholar
  20. 20.
    Chan LC, Kwong YL, Lie AK, et al. A case of Philadelphia-negative, M-BCR rearranged eosinophilic leukaemia with trisomy 8 localized by in situ hybridization. Leukemia 1994 Jan; 8 (1): 195–8PubMedGoogle Scholar
  21. 21.
    Singh RP, Emmatty JG, Bhama T. Hodgkin’s lymphoma presenting as hypereosinophilia syndrome. J Assoc Physicians India 1984 Jun; 32 (6): 528–9PubMedGoogle Scholar
  22. 22.
    Manthorpe R, Egeberg J, Hesselvik M, et al. Unique eosinophil granules in a case of T-cell lymphoma. Scand J Haematol 1977 Aug; 19 (2): 129–44PubMedGoogle Scholar
  23. 23.
    Kimura H, Abe R, Shiga Y, et al A case of acute myelogenous leukaemia associated with eosinophilia: cytogenetic study of eosinophilic colonies showing the origin of the normal clone. Acta Haematol 1987; 77 (1): 15–9PubMedGoogle Scholar
  24. 24.
    Spitzer G, Garson OM. Lymphoblastic leukemia with marked eosinophilia: a report of two cases. Blood 1973 Sep; 42 (3): 377–84PubMedGoogle Scholar
  25. 25.
    Koeffler HP, Levine AM, Sparkes M, et al. Chronic myelocytic leukemia: eosinophils involved in the malignant clone. Blood 1980 Jun; 55 (6): 1063–5PubMedGoogle Scholar
  26. 26.
    Miranda RN, Esparza AR, Sambandam S, et al. Systemic mast cell disease presenting with peripheral blood eosinophilia. Hum Pathol 1994 Jul; 25 (7): 727–30PubMedGoogle Scholar
  27. 27.
    Isaacson NH, Rapoport P. Eosinophilia in malignant tumors: its significance. Ann Intern Med 1946 Dec; 25 (6): 893–902PubMedGoogle Scholar
  28. 28.
    Viola MV, Chung E, Mukhopadhyay MG. Eosinophilia and metastatic carcinoma. Med Ann Dist Columbia 1972 Jan; 41 (1): 1–3PubMedGoogle Scholar
  29. 29.
    Lieske TR, Sunderrajan EV, Passamonte PM. Bronchoalveolar lavage and technetium-99m glucoheptonate imaging in chronic eosinophilic pneumonia. Chest 1984 Feb; 85 (2): 282–4PubMedGoogle Scholar
  30. 30.
    McEvoy JD, Donald KJ, Edwards RL. Immunoglobulin levels and electron microscopy in eosinophilic pneumonia. Am J Med 1978 Mar; 64 (3): 529–36PubMedGoogle Scholar
  31. 31.
    Allen JN, Pacht ER, Gadek JE, et al. Acute eosinophilic pneumonia as a reversible cause of noninfectious respiratory failure. N Engl J Med 1989 Aug 31; 321 (9): 569–74PubMedGoogle Scholar
  32. 32.
    Umeki S. Reevaluation of eosinophilic pneumonia and its diagnostic criteria. Arch Intern Med 1992 Sep; 152 (9): 1913–9PubMedGoogle Scholar
  33. 33.
    Neva FA, Ottesen EA. Tropical (filarial) eosinophilia. N Engl J Med 1978 May 18; 298 (20): 1129–31PubMedGoogle Scholar
  34. 34.
    Farnam J, Alperin JB, Ladoulis C, et al. Mononeuritis multiplex and eosinophilia in a patient with asthma. Ann Allergy 1985 Jul; 55(1): 14–5, 33-5PubMedGoogle Scholar
  35. 35.
    Rose GA. The natural history of polyarteritis. BMJ 1957 Nov 16; 2: 1148–52PubMedGoogle Scholar
  36. 36.
    Lanham JG, Elkon KB, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984 Mar; 63 (2): 65–81Google Scholar
  37. 37.
    Chen KR, Su WP, Pittelkow MR, et al. Eosinophilic vasculitis in connective tissue disease. J Am Acad Dermatol 1996 Aug; 35 (2): 173–82PubMedGoogle Scholar
  38. 38.
    Nutman TB, Ottesen EA, Cohen SG. Eosinophilia and eosinophil-related disorders. In: Middleton Jr E, Reed CE, Ellis EF, et al., editors. Allergy: principles and practice. 3rd ed. Vol 2. St. Louis: CV Mosby Company, 1988: 868Google Scholar
  39. 39.
    Nakanishi K, Kaneko T, Yano F, et al Marked eosinophilia induced by nafamostat mesilate, an anticoagulant in a hemo-dialysis patient. Nephron 1992; 62 (1): 97–9PubMedGoogle Scholar
  40. 40.
    van Haelst Pisani C, Kovach JS, Kita H, et al. Administration of interleukin-2 (IL-2) results in increased plasma concentrations of IL-5 and eosinophilia in patients with cancer. Blood 1991 Sep 15; 78 (6): 1538–44PubMedGoogle Scholar
  41. 41.
    Freundlich B, Werth VP, Rook AH, et al. L-tryptophan ingestion associated with eosinophilic fasciitis but not progressive systemic sclerosis. Ann Intern Med 1990 May 15; 112 (10): 758–62PubMedGoogle Scholar
  42. 42.
    Cello JP. Eosinophilic gastroenteritis — a complex disease entity. Am J Med 1979 Dec; 67 (6): 1097–104PubMedGoogle Scholar
  43. 43.
    Johnstone JM, Morson BC. Eosinophilic gastroenteritis. Histopathology 1978 Sep; 2 (5): 335–48PubMedGoogle Scholar
  44. 44.
    Gleich GJ, Schroeter AL, Marcoux JP, et al. Episodic angioedema associated with eosinophilia. N Engl J Med 1984 Jun 21; 310 (25): 1621–6PubMedGoogle Scholar
  45. 45.
    Bushkell LL, Jordon RE. Bullous pemphigoid: a cause of peripheral blood eosinophilia. J Am Acad Dermatol 1983 May; 8 (5): 648–51PubMedGoogle Scholar
  46. 46.
    Chang YT, Liu HN, Wong CK. Bullous pemphigoid — a report of 86 cases from Taiwan. Clin Exp Dermatol 1996 Jan; 21 (1): 20–2PubMedGoogle Scholar
  47. 47.
    Kuo TT, Shih LY, Chan HL. Kimura’s disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia. Am J Surg Pathol 1988 Nov; 12 (11): 843–54PubMedGoogle Scholar
  48. 48.
    Fisher GB, Greer KE, Cooper PH. Eosinophilic cellulitis (Wells’ syndrome). Int J Dermatol 1985 Mar; 24 (2): 101–7PubMedGoogle Scholar
  49. 49.
    Peters MS, Schroeter AL, Gleich GJ. Immunofluorescence identification of eosinophil granule major basic protein in the flame figures of Wells’ syndrome. Br J Dermatol 1983 Aug; 109 (2): 141–8PubMedGoogle Scholar
  50. 50.
    Martin RW, Duffy J, Engel AG, et al. The clinical spectrum of the eosinophilia-myalgia syndrome associated with L-tryptophan ingestion: clinical features in 20 patients and aspects of pathophysiology. Ann Intern Med 1990 Jul 15; 113 (2): 124–34PubMedGoogle Scholar
  51. 51.
    Kaufman LD, Finn Jr AF, Seidman RJ, et al. Eosinophilic neuritis, perimyositis, and vasculitis associated with ingestion of L-tryptophan. J Rheumatol 1990 Jun; 17 (6): 795–800PubMedGoogle Scholar
  52. 52.
    Clauw DJ, Crofford LJ. Eosinophilic rheumatic disorders. Rheum Dis Clin North Am 1995 Feb; 21 (1): 231–46PubMedGoogle Scholar
  53. 53.
    Michet Jr CJ, Doyle JA, Ginsburg WW. Eosinophilic fasciitis: report of 15 cases. Mayo Clin Proc 1981 Jan; 56 (1): 27–34PubMedGoogle Scholar
  54. 54.
    Parrillo JE, Fauci AS, Wolff SM. The hypereosinophilic syndrome: dramatic response to therapeutic intervention. Trans Assoc Am Physicians 1977; 90: 135–44PubMedGoogle Scholar
  55. 55.
    Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood 1994 May 15; 83 (10): 2759–79PubMedGoogle Scholar
  56. 56.
    Schooley RT, Parrillo JE, Wolff SM, et al. Management of the idiopathic hypereosinophilic syndrome. In: Mahmoud AAF, Austen KF, Stolper SA, editors. Eosinophils in health and disease. New York: Grune & Stratton, 1980: 323–43Google Scholar
  57. 57.
    Confalonieri M, Tacconi F, De Amici M, et al. Benign idiopathic hypereosinophilia: a feeble masquerader or a smouldering form of the hypereosinophilic syndrome? Haematologica 1995 Jan–Feb; 80 (1): 50–3PubMedGoogle Scholar
  58. 58.
    Harley JB, Fauci AS, Gralnick HR. Noncardiovascular findings associated with heart disease in the idiopathic hypereosinophilic syndrome. Am J Cardiol 1983 Aug; 52 (3): 321–4PubMedGoogle Scholar
  59. 59.
    Spry CJ, Davies J, Tai PC, et al. Clinical features of fifteen patients with the hypereosinophilic syndrome. Q J Med 1983 Winter; 52 (205): 1–22PubMedGoogle Scholar
  60. 60.
    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. N Engl J Med 1994 Feb 24; 330 (8): 535–8PubMedGoogle Scholar
  61. 61.
    Takai K, Sanada M. Hypereosinophilic syndrome evolving to acute lymphoblastic leukemia. Int J Hematol 1991 Jun; 54 (3): 231–9PubMedGoogle Scholar
  62. 62.
    Needleman SW, Mane SM, Gutheil JC, et al Hypereosinophilic syndrome with evolution to myeloproliferative disorder: temporal relationship to loss of Y chromosome and c-N-ras activation. Hematol Pathol 1990; 4 (3): 149–55PubMedGoogle Scholar
  63. 63.
    Kim CJ, Park SH, Chi JG. Idiopathic hypereosinophilic syndrome terminating as disseminated T-cell lymphoma. Cancer 1991 Feb 15; 67 (4): 1064–9PubMedGoogle Scholar
  64. 64.
    Higuchi W, Koike T, Ihizumi T, et al Hypereosinophilic syndrome terminating in acute myelogenous leukemia. Acta Haematol 1993; 90 (3): 165–6PubMedGoogle Scholar
  65. 65.
    Dourov N, Cauchie C, de Maertelaere-Laurent E, et al Myelome et hypereosinophilie: reaction leucemoide a eosin-ophiles associee a un myelome. Nouv Rev Fr Hematol 1981; 23 (4): 227–34PubMedGoogle Scholar
  66. 66.
    Ishida T, Morita M, Kawanami M, et al. A case of hypereosinophilic syndrome associated with eosinophilic endocarditis. Kokyu To Junkan 1989 Aug; 37 (8): 909–13PubMedGoogle Scholar
  67. 67.
    Bush RK, Geller M, Busse WW, et al. Response to corticosteroids in the hypereosinophilic syndrome: association with increased serum IgE levels. Arch Intern Med 1978 Aug; 138 (8): 1244–6PubMedGoogle Scholar
  68. 68.
    Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med 1978 Aug; 89 (2): 167–72PubMedGoogle Scholar
  69. 69.
    Rizzari C, Conter V, D’Angelo P, et al. Efficacy of prolonged low-dose steroid treatment in a child with idiopathic hypereosinophilic syndrome: a case report [letter]. Pediatr Hematol Oncol 1995 Mar–Apr; 12 (2): 209–12PubMedGoogle Scholar
  70. 70.
    Edwards D, Wald JA, Dobozin BS, et al. Troleandomycin and methylprednisolone for treatment of the hypereosinophilic syndrome [letter]. N Engl J Med 1987 Aug 27; 317 (9): 573–4PubMedGoogle Scholar
  71. 71.
    Wallen N, Kita H, Weiler D, et al. Glucocorticoids inhibit cytokine-mediated eosinophil survival. J Immunol 1991 Nov 15; 147 (10): 3490–5PubMedGoogle Scholar
  72. 72.
    Lamas AM, Leon OG, Schleimer RP. Glucocorticoids inhibit eosinophil responses to granulocyte-macrophage colony-stimulating factor. J Immunol 1991 Jul 1; 147 (1): 254–9PubMedGoogle Scholar
  73. 73.
    Cox G, Ohtoshi T, Vancheri C, et al. Promotion of eosinophil survival by human bronchial epithelial cells and its modulation by steroids. Am J Respir Cell Mol Biol 1991 Jun; 4 (6): 525–31PubMedGoogle Scholar
  74. 74.
    Her E, Frazer J, Austen KF, et al. Eosinophil hematopoietins antagonize the programmed cell death of eosinophils: cytokine and glucocorticoid effects on eosinophils maintained by endothelial cell-conditioned medium. J Clin Invest 1991 Dec; 88 (6): 1982–7PubMedGoogle Scholar
  75. 75.
    Hallsworth MP, Litchfield TM, Lee TH. Glucocorticoids inhibit granulocyte-macrophage colony-stimulating factor-1 and interleukin-5 enhanced in vitro survival of human eosinophils. Immunology 1992 Feb; 75 (2): 382–5PubMedGoogle Scholar
  76. 76.
    Schrezenmeier H, Thome SD, Tewald F, et al. Interleukin-5 is the predominant eosinophilopoietin produced by cloned T lymphocytes in hypereosinophilic syndrome. Exp Hematol 1993 Feb; 21 (2): 358–65PubMedGoogle Scholar
  77. 77.
    Fang J, Viksman MY, Ebisawa M, et al. Increased circulating levels of interleukin-5 in a case of steroid-resistant hypereosinophilic syndrome with ileal involvement. J Allergy Clin Immunol 1994 Jul; 94 (1): 129–31PubMedGoogle Scholar
  78. 78.
    Butterfield JH, Leiferman KM, Abrams J, et al. Elevated serum levels of interleukin-5 in patients with the syndrome of episodic angioedema and eosinophilia. Blood 1992 Feb 1; 79 (3): 688–92PubMedGoogle Scholar
  79. 79.
    Fruehauf S, Fiehn C, Haas R, et al Sustained remission of idiopathic hypereosinophilic syndrome following alpha-interferon therapy. Acta Haematol 1993; 89 (2): 91–3PubMedGoogle Scholar
  80. 80.
    Butterfield JH, Ackerman SJ, Weiler D, et al. Effects of glucocorticoids on eosinophil colony growth. J Allergy Clin Immunol 1986 Sep; 78 (3 Pt 1): 450–7PubMedGoogle Scholar
  81. 81.
    Bjornson BH, Harvey JM, Rose L. Differential effect of hydrocortisone on eosinophil and neutrophil proliferation. J Clin Invest 1985 Sep; 76 (3): 924–9PubMedGoogle Scholar
  82. 82.
    Journey LJ, Burdman J, George P. Ultrastructural studies on tissue culture cells treated with vincristine (NSC-67574). Cancer Chemother Rep 1968 Jun; 52 (4): 509–17PubMedGoogle Scholar
  83. 83.
    Van Slyck EJ, Adamson TC III. Acute hypereosinophilic syndrome: successful treatment with vincristine, cytarabine, and prednisone. JAMA 1979 Jul 13; 242 (2): 175–6PubMedGoogle Scholar
  84. 84.
    Sakamoto K, Erdreich-Epstein A, deClerck Y, et al. Prolonged clinical response to vincristine treatment in two patients with idiopathic hypereosinophilic syndrome. Am J Pediatr Hematol Oncol 1992 Nov; 14 (4): 348–51PubMedGoogle Scholar
  85. 85.
    Marshall GM, White L. Effective therapy for a severe case of the idiopathic hypereosinophilic syndrome. Am J Pediatr Hematol Oncol 1989 Summer; 11 (2): 178–83PubMedGoogle Scholar
  86. 86.
    Wynn SR, Sachs MI, Keating MU, et al. Idiopathic hypereosinophilic syndrome in a 5 ½-month-old infant. J Pediatr 1987 Jul; 111 (1): 94–7PubMedGoogle Scholar
  87. 87.
    Cofrancesco E, Cortellaro M, Pogliani E, et al Response to vincristine treatment in a case of idiopathic hypereosinophilic syndrome with multiple clinical manifestations. Acta Haematol 1984; 72 (1): 21–5PubMedGoogle Scholar
  88. 88.
    Clarkson BD, Dowling MD, Gee TS, et al. Treatment of acute leukemia in adults. Cancer 1975 Aug; 36 (2): 775–95PubMedGoogle Scholar
  89. 89.
    Eakin DL, Gill DP, Weiss GB. Response of hypereosinophilic syndrome to 6-thioguanine and cytarabine. Cancer Treat Rep 1982 Mar; 66 (3): 545–7PubMedGoogle Scholar
  90. 90.
    Smit AJ, van Essen LH, de Vries EG. Successful long-term control of idiopathic hypereosinophilic syndrome with etoposide. Cancer 1991 Jun 1; 67 (11): 2826–7PubMedGoogle Scholar
  91. 91.
    Bourrat E, Lebbe C, Calvo F. Etoposide for treating the hypereosinophilic syndrome [letter]. Ann Intern Med 1994 Dec 1; 121 (11): 899–900PubMedGoogle Scholar
  92. 92.
    Raghavachar A, Fleischer S, Frickhofen N, et al. T lymphocyte control of human eosinophilic granulopoiesis. Clonal analysis in an idiopathic hypereosinophilic syndrome. J Immunol 1987 Dec 1; 139 (11): 3753–8PubMedGoogle Scholar
  93. 93.
    Shupack JL, Kenny C, Jondreau L, et al Decreased peripheral blood eosinophil counts in severe psoriatic patients treated with low-dose cyclosporine A. Dermatology 1992; 185 (3): 202–4PubMedGoogle Scholar
  94. 94.
    Zabel P, Schlaak M. Cyclosporin for hypereosinophilic syndrome. Ann Hematol 1991 Jun; 62 (6): 230–1PubMedGoogle Scholar
  95. 95.
    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. J Immunol 1983 Sep; 131 (3): 1300–5PubMedGoogle Scholar
  96. 96.
    Valerius T, Repp R, Kalden JR, et al. Effects of IFN on human eosinophils in comparison with other cytokines. A novel class of eosinophil activators with delayed onset of action. J Immunol 1990 Nov 1; 145 (9): 2950–8PubMedGoogle Scholar
  97. 97.
    Schandene L, Del Prete GF, Cogan E, et al. Recombinant interferon-alpha selectively inhibits the production of interleukin-5 by human CD4+ T cells. J Clin Invest 1996 Jan 15; 97 (2): 309–15PubMedGoogle Scholar
  98. 98.
    Murphy PT, Fennelly DF, Stuart M, et al. Alfa-interferon in a case of hypereosinophilic syndrome. Br J Haematol 1990 Aug; 75 (4): 619–20PubMedGoogle Scholar
  99. 99.
    Zielihski RM, Lawrence WD. Interferon-alpha for the hypereosinophilic syndrome. Ann Intern Med 1990 Nov 1; 113 (9): 716–8Google Scholar
  100. 100.
    Busch FW, Schmidt H, Steinke B. Alpha-interferon for the hypereosinophilic syndrome [letter]. Ann Intern Med 1991 Feb 15; 114 (4): 338–9PubMedGoogle Scholar
  101. 101.
    Bockenstedt PL, Santinga JT, Boiling SF. Alpha-interferon treatment for idiopathic hypereosinophilic syndrome. Am J Hematol 1994 Mar; 45 (3): 248–51PubMedGoogle Scholar
  102. 102.
    Canonica GW, Passalacqua G, Pronzato C, et al. Effective long-term alpha-interferon treatment for hypereosinophilic syndrome. J Allergy Clin Immunol 1995 Jul; 96 (1): 131–3PubMedGoogle Scholar
  103. 103.
    Coutant G, Bletry O, Prin L, et al Traitement des syndromes hypereosinophiliques a expression myeloproliferative par l’association hydroxyuree-interferon alpha: a propos de 7 observations. Ann Med Interne Paris 1993; 144 (4): 243–50PubMedGoogle Scholar
  104. 104.
    Butterfield JH, Gleich GJ. Interferon-alpha treatment of six patients with the idiopathic hypereosinophilic syndrome. Ann Intern Med 1994 Nov 1; 121 (9): 648–53PubMedGoogle Scholar
  105. 105.
    Kobayashi M, Katayama T, Ochiai S, et al. Interferon-alpha therapy in the myeloproliferative variants of hypereosinophilic syndrome. Rinsho Ketsueki 1993 Mar; 34 (3): 367–72PubMedGoogle Scholar
  106. 106.
    Schandene L, Ferster A, Mascart-Lemone F, et al. T helper type 2-like cells and therapeutic effects of interferon-gamma in combined immunodeficiency with hypereosinophilia (Omenn’s syndrome). Eur J Immunol 1993 Jan; 23 (1): 56–60PubMedGoogle Scholar
  107. 107.
    Hanifin JM, Schneider LC, Leung DY, et al. Recombinant interferon gamma therapy for atopic dermatitis. J Am Acad Dermatol 1993 Feb; 28 (2 Pt 1): 189–97PubMedGoogle Scholar
  108. 108.
    Prin L, Plumas J, Gruart V, et al. Elevated serum levels of soluble interleukin-2 receptor: a marker of disease activity in the hypereosinophilic syndrome. Blood 1991 Nov 15; 78 (10): 2626–32PubMedGoogle Scholar
  109. 109.
    Kantarjian HM, Smith TL, O’Brien S, et al. Prolonged survival in chronic myelogenous leukemia after cytogenetic response to interferon-alpha therapy. Ann Intern Med 1995 Feb 15; 122 (4): 254–61PubMedGoogle Scholar
  110. 110.
    Quiquandon I, Claisse JF, Capiod JC, et al. Alpha-interferon and hypereosinophilic syndrome with trisomy 8: karyotypic remission [letter]. Blood 1995 Apr 15; 85 (8): 2284–5PubMedGoogle Scholar
  111. 111.
    van den Anker-Lugtenburg PJ, van’t Veer MB. Alpha-interferon in a case of hypereosinophilic syndrome [letter]. Br J Haematol 1991 Feb; 77 (2): 258PubMedGoogle Scholar
  112. 112.
    Blanquer A, Lopez A, Tarin F, et al. Alpha interferon for hypereosinophilic syndrome [letter]. Am J Hematol 1994 Dec; 47 (4): 332–3PubMedGoogle Scholar
  113. 113.
    Sato H, Danbara M, Tamura M, et al. Eosinophilic leukaemia with a t(2; 5) (p23; q35) translocation. Br J Haematol 1994 Jun; 87 (2): 404–6PubMedGoogle Scholar
  114. 114.
    Hendrick A. Psychosis during interferon in eosinophilic leukaemia. Clin Lab Haematol 1994 Sep; 16 (3): 295–6PubMedGoogle Scholar
  115. 115.
    Hansen PB, Johnsen HE, Hippe E. Hypereosinophilic syndrome treated with alpha-interferon and granulocyte colony-stimulating factor but complicated by nephrotoxicity. Am J Hematol 1993 May; 43 (1): 66–8PubMedGoogle Scholar
  116. 116.
    Chambers LA, Leonard SS, Whatmough AE, et al. Management of hypereosinophilic syndrome with chronic plasma- and leukapheresis. Prog Clin Biol Res 1990; 337: 83–5PubMedGoogle Scholar
  117. 117.
    Pineda AA, Brzica Jr SM, Taswell HF. Continuous- and semi-continuous-flow blood centrifugation systems: therapeutic applications, with plasma-, platelet, lympha-, and eosinapheresis. Transfusion 1977 Sep–Oct; 17 (5): 407–16PubMedGoogle Scholar
  118. 118.
    Ellman L, Miller L, Rappeport J. Leukopheresis therapy of a hypereosinophilic disorder. JAMA 1974 Nov 18; 230 (7): 1004–5PubMedGoogle Scholar
  119. 119.
    Archimbaud E, Guyotat D, Guillaume C, et al. Hypereosinophilic syndrome with multiple organ dysfunction treated by al-logeneic bone marrow transplantation. Am J Hematol 1988 Apr; 27 (4): 302–3PubMedGoogle Scholar
  120. 120.
    Fukushima T, Kuriyama K, Ito H, et al. Successful bone marrow transplantation for idiopathic hypereosinophilic syndrome. Br J Haematol 1995 May; 90 (1): 213–5PubMedGoogle Scholar
  121. 121.
    Sigmund DA, Flessa HC. Hypereosinophilic syndrome: successful allogeneic bone marrow transplantation. Bone Marrow Transplant 1995 Apr; 15 (4): 647–8PubMedGoogle Scholar
  122. 122.
    Esteva-Lorenzo FJ, Meehan KR, Spitzer TR, et al. Allogeneic bone marrow transplantation in a patient with hypereosinophilic syndrome. Am J Hematol 1996 Feb; 51 (2): 164–5PubMedGoogle Scholar
  123. 123.
    Kazmierowski JA, Chusid MJ, Parrillo JE, et al. Dermatologic manifestations of the hypereosinophilic syndrome. Arch Dermatol 1978 Apr; 114 (4): 531–5PubMedGoogle Scholar
  124. 124.
    Wemmer U, Thiele B, Steigleder GK. Hypereosinophilie-Syndrom (HES) — erfolgreiche PUVA-Therapie. Hautarzt 1988 Jan; 39(1): 42–4PubMedGoogle Scholar
  125. 125.
    van den Hoogenband HM, van den Berg WH, van Diggelen MW. PUVA therapy in the treatment of the skin lesions of the hypereosinophilic syndrome [letter]. Arch Dermatol 1985 Apr; 121 (4): 450PubMedGoogle Scholar
  126. 126.
    Newton JA, Singh AK, Greaves MW, et al. Aquagenic pruritus associated with the idiopathic hypereosinophilic syndrome. Br J Dermatol 1990 Jan; 122 (1): 103–6PubMedGoogle Scholar
  127. 127.
    May LP, Kelly J, Sanchez M. Hypereosinophilic syndrome with unusual cutaneous manifestations in two men with HIV infection. J Am Acad Dermatol 1990 Aug; 23 (2 Pt 1): 202–4PubMedGoogle Scholar
  128. 128.
    Nir MA, Westfried M Hypereosinophilic dermatitis: a distinct manifestation of the hypereosinophilic syndrome with response to dapsone. Dermatologica 1981; 162 (6): 444–50PubMedGoogle Scholar
  129. 129.
    Wood ML, Slater DN. Cutaneous manifestations of the hypereosinophilic syndrome treated with sodium cromoglycate. Clin Exp Dermatol 1984 Mar; 9 (2): 139–42PubMedGoogle Scholar
  130. 130.
    Leiferman KM, O’Duffy JD, Perry HO, et al. Recurrent incapacitating mucosal ulcerations: a prodrome of the hypereosinophilic syndrome. JAMA 1982 Feb 19; 247 (7): 1018–20PubMedGoogle Scholar
  131. 131.
    Akahoshi M, Hoshino S, Teramura M, et al. Recurrent oral and genital ulcers as the prodrome of the hypereosinophilic syndrome: report of a probable case. Nippon Naika Gakkai Zasshi 1987 Mar; 76 (3): 421–4PubMedGoogle Scholar
  132. 132.
    Butterfield JH, Gleich GJ, Gillham Jr RA, et al. Healing of recurrent incapacitating mucosal ulcers of the hypereosinophilic syndrome (HES) with interferon alfa 2B [abstract]. J Allergy Clin Immunol 1993; 91: 317Google Scholar
  133. 133.
    Gleich GJ, Loegering DA, Kueppers F, et al. Physiochemical and biological properties of the major basic protein from guinea pig eosinophil granules. J Exp Med 1974 Aug 1; 140 (2): 313–32PubMedGoogle Scholar
  134. 134.
    Hanowell ST, Kim YD, Rattan V, et al. Increased heparin requirement with hypereosinophilic syndrome. Anesthesiology 1981 Oct; 55 (4): 450–2PubMedGoogle Scholar
  135. 135.
    Slungaard A, Vercellotti GM, Tran T, et al. Eosinophil cationic granule proteins impair thrombomodulin function: a potential mechanism for thromboembolism in hypereosinophilic heart disease. J Clin Invest 1993 Apr; 91 (4): 1721–30PubMedGoogle Scholar
  136. 136.
    Rohrbach MS, Wheatley CL, Slifman NR, et al. Activation of platelets by eosinophil granule proteins. J Exp Med 1990 Oct 1; 172(4): 1271–4PubMedGoogle Scholar
  137. 137.
    Venge P, Dahl R, Hallgren R. Enhancement of factor XII dependent reactions by eosinophil cationic protein. Thromb Res 1979; 14 (4-5): 641–9PubMedGoogle Scholar
  138. 138.
    Dahl R, Venge P. Enhancement of urokinase-induced plasminogen activation by the cationic protein of human eosinophil granulocytes. Thromb Res 1979; 14 (4-5): 599–608PubMedGoogle Scholar
  139. 139.
    Pincus SH, Ramesh KS, Wyler DJ. Eosinophils stimulate fibroblast DNA synthesis. Blood 1987 Aug; 70 (2): 572–4PubMedGoogle Scholar
  140. 140.
    Lombardi C, Rusconi C, Faggiano P, et al. Successful reduction of endomyocardial fibrosis in a patient with idiopathic hypereosinophilic syndrome: a case report. Angiology 1995 Apr; 46 (4): 345–51PubMedGoogle Scholar
  141. 141.
    Solley GO, Maldonado JE, Gleich GJ, et al. Endomyocardio-pathy with eosinophilia. Mayo Clin Proc 1976 Nov; 51 (11): 697–708PubMedGoogle Scholar
  142. 142.
    Wolz DE, Granato JE, Giles HR, et al. A unique chromosomal abnormality in idiopathic hypereosinophilic syndrome presenting with cardiac involvement. Am Heart J 1993 Jul; 126 (1): 246–8PubMedGoogle Scholar
  143. 143.
    Parrillo JE, Borer JS, Henry WL, et al. The cardiovascular manifestations of the hypereosinophilic syndrome: prospective study of 26 patients, with review of the literature. Am J Med 1979 Oct; 67 (4): 572–82PubMedGoogle Scholar
  144. 144.
    Davies J, Spry CJ, Sapsford R, et al. Cardiovascular features of 11 patients with eosinophilic endomyocardial disease. Q J Med 1983 Winter; 52 (205): 23–39PubMedGoogle Scholar
  145. 145.
    Inoue K, Saida K, Yamashiro M, et al. Mitral valve replacement in idiopathic hypereosinophilic syndrome. Nippon Kyobu Geka Gakkai Zasshi 1991 Nov; 39 (11): 2068–73PubMedGoogle Scholar
  146. 146.
    Tanino M, Kitamura K, Ohta G, et al. Hypereosinophilic syndrome with extensive myocardial involvement and mitral valve thrombus instead of mural thrombi. Acta Pathol Jpn 1983 Nov; 33 (6): 1233–42PubMedGoogle Scholar
  147. 147.
    Tai PC, Ackerman SJ, Spry CJ, et al. Deposits of eosinophil granule proteins in cardiac tissues of patients with eosinophilic endomyocardial disease. Lancet 1987 Mar 21; I (8534): 643–7Google Scholar
  148. 148.
    Take M, Sekiguchi M, Hiroe M, et al. Clinical spectrum and endomyocardial biopsy findings in eosinophilic heart disease. Heart Vessels Suppl 1985; 1: 243–9PubMedGoogle Scholar
  149. 149.
    Fournial G, Schlanger R, Berthoumieu F, et al. Surgery for cardiac complications caused by endocardial mural fibrin deposits in a hypereosinophilic syndrome. Circulation 1982 May; 65 (5): 1010–4PubMedGoogle Scholar
  150. 150.
    Davies J, Sapsford R, Brooksby I, et al. Successful surgical treatment of two patients with eosinophilic endomyocardial disease. Br Heart J 1981 Oct; 46 (4): 438–45PubMedGoogle Scholar
  151. 151.
    Harley JB, McIntosh CL, Kirklin JJ, et al. Atrioventricular valve replacement in the idiopathic hypereosinophilic syndrome. Am J Med 1982 Jul; 73 (1): 77–81PubMedGoogle Scholar
  152. 152.
    Blake DP, Palmer TE, Olinger GN. Mitral valve replacement in idiopathic hypereosinophilic syndrome. J Thorac Cardiovasc Surg 1985 Apr; 89 (4): 630–2PubMedGoogle Scholar
  153. 153.
    Boustany Jr CW, Murphy GW, Hicks Jr GL. Mitral valve replacement in idiopathic hypereosinophilic syndrome. Ann Thorac Surg 1991 Jun; 51 (6): 1007–9PubMedGoogle Scholar
  154. 154.
    Smith MD, Metcalfe M, DeMaria AN, et al. Hypereosinophilic syndrome resulting in aortic and mitral stenosis: a case requiring double valve replacement. Am Heart J 1989 Feb; 117 (2): 475–9PubMedGoogle Scholar
  155. 155.
    Hendren WG, Jones EL, Smith MD. Aortic and mitral valve replacement in idiopathic hypereosinophilic syndrome. Ann Thorac Surg 1988 Nov; 46 (5): 570–1PubMedGoogle Scholar
  156. 156.
    Sakakibara Y, Okamura K, Sakurai J, et al Successful case of double-valve replacement for aortic steno-regurgitation and mitral stenosis combined with thrombocytopenia and eosinophilia. Kyobu Geka 1982; 35 (7): 558–62PubMedGoogle Scholar
  157. 157.
    Wood AE, Boyle D, O’Hara MD, et al. Mitral annuloplasty in endomyocardial fibrosis: an alternative to valve replacement. Ann Thorac Surg 1982 Oct; 34 (4): 446–51PubMedGoogle Scholar
  158. 158.
    Bell JA, Jenkins BS, Webb-Peploe MM. Clinical, haemodynamic, and angiographic findings in Loffler’s eosinophilic endocarditis. Br Heart J 1976 Jun; 38 (6): 541–8PubMedGoogle Scholar
  159. 159.
    Weyman AE, Rankin R, King H. Loeffler’s endocarditis presenting as mitral and tricuspid stenosis. Am J Cardiol 1977 Sep; 40 (3): 438–44PubMedGoogle Scholar
  160. 160.
    Cameron J, Radford DJ, Howell J, et al. Hypereosinophilic heart disease. Med J Aust 1985 Oct 28; 143 (9): 408–10PubMedGoogle Scholar
  161. 161.
    Dubost C, Chapelon C, Deloche A, et al. Chirurgie des fibroses endomyocardiques: a propos de 32 cas. Arch Mal Coeur Vaiss 1990 Apr; 83 (4): 481–6PubMedGoogle Scholar
  162. 162.
    Adams HW, Mainz DL. Eosinophilic ascites: a case report and review of the literature. Am J Dig Dis 1977 Jan; 22 (1): 40–2PubMedGoogle Scholar
  163. 163.
    Arvay A, Lengyel M, Meszaros R, et al. Surgical experience with thrombotic and fibrotic forms of non-tropical eosinophilic endomyocardial disease. Thorac Cardiovasc Surg 1985 Oct; 33 (5): 314–6PubMedGoogle Scholar
  164. 164.
    Ikaheimo MJ, Karkola PJ, Takkunen JT. Surgical treatment of Loffler’s eosinophilic endocarditis. Br Heart J 1981 Jun; 45 (6): 729–32PubMedGoogle Scholar

Copyright information

© Adis International Limited 1997

Authors and Affiliations

  • Joseph H. Butterfield
    • 1
  1. 1.Division of Allergy/Outpatient Infectious Disease and Internal MedicineMayo Clinic and Mayo FoundationRochesterUSA

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