The Indian Journal of Pediatrics

, Volume 80, Issue 2, pp 124–127

Idiopathic Hypereosinophilic Syndrome in Children: 3 Cases with Review of Literature

Original Article

Abstract

Objectives

To highlight the varied clinical manifestations and management of idiopathic hypereosinophilic syndrome, a rare disorder in children.

Methods

Retrospective review of case records of 3 patients who were diagnosed to have idiopathic hypereosinophilic syndrome (IHES) in a tertiary referral centre between 1997 and 2010 was performed. These 3 children presented with different symptoms and the first case had cardiac involvement. All had very high absolute eosinophil count (cells/mm3) 43,206, 9,082, 2,925, respectively. Diagnosis was confirmed by bone marrow biopsy in all three cases and supported by liver biopsy in the first two cases and inguinal lymphnode biopsy in the last case. All 3 children responded to treatment with steroids, with only second patient requiring hydroxyurea to control disease.

Results

Case 1 and 2 are on follow up for 13 y and 10 y respectively and both are asymptomatic. Case 3 expired due to sepsis 1 mo after diagnosis.

Conclusions

Treatment with steroids with or without hydroxyurea gave good response in all 3 cases. Hence, they still remain the gold standard for the treatment of IHES in children.

Keywords

Idiopathic hypereosinophilic syndrome Children Steroids 

References

  1. 1.
    Hardy WR, Anderson RE. The hypereosinophilic syndromes. Ann Intern Med. 1968;68:1220–9.PubMedGoogle Scholar
  2. 2.
    Chusid MJ, Dale DC, West BC, Wolf SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54:1–27.CrossRefGoogle Scholar
  3. 3.
    Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994;83:2759–79.PubMedGoogle Scholar
  4. 4.
    Boyce JA, Owen WF. Idiopathic hypereosinophilic syndrome. In: Kaplan AP, ed. Allergy. Philadelphia: WB Saunders; 1997. pp. 861–75.Google Scholar
  5. 5.
    Alfaham MA, Ferguson SD, Shira B. The idiopathic hypereosinophilic syndrome. Arch Dis Child. 1987;62:601–13.PubMedCrossRefGoogle Scholar
  6. 6.
    Salm CM, Clair NE, Lustig JV, Samyn MM. Cardiac manifestations from non-FIP1L1-PDGFRα-associated hypereosinophilic syndrome in a 13-year-old African American boy. J Allergy (Cairo). 2009;2009:804910.Google Scholar
  7. 7.
    Weaver DF, Heffernan LP, Purdy RA. Eosinophil-induced neurotoxicity: axonal neuropathy, cerebral infarction and dementia. Neurology. 1988;38:144–6.PubMedCrossRefGoogle Scholar
  8. 8.
    Pérez PJ, Gomez Papí A, Tarrés Roure A, Llorente Cabrera A. Idiopathic hypereosinophilic syndrome in a 6-year-old child. An Esp Pediatr. 1990;33:69–70.Google Scholar
  9. 9.
    Win SR, Sachs M, Keating MUS. Idiopathic hypereosinophilic syndrome in a 51/2-month-old infant. J Pediatr. 1987;111:94–7.CrossRefGoogle Scholar
  10. 10.
    FauciAS HJB, Roberts WC, Ferrans VJ, Gralnick HR, Bjornson BH. NIH Conference. The idiopathic hypereosinophilic syndrome: Clinical, pathophysiologic and therapeutic considerations. Ann Int Med. 1982;97:78–92.Google Scholar
  11. 11.
    Smit AJ, van Essen LH, de Vries EG. Successful long-term control of idiopathic hypereosinophilic syndrome with etoposide. Cancer. 1991;67:2826–7.PubMedCrossRefGoogle Scholar
  12. 12.
    Sakamoto K, Erdreich E, de Clerck Y, Coates T. Prolonged clinical response to vincristine treatment in two patients with idiopathic hypereosinophilic syndrome. Am J Pediatr Hematol Oncol. 1992;14:348–51.PubMedCrossRefGoogle Scholar
  13. 13.
    Bockenstedt PL, Santinga JT, Bolling SF. Alpha-interferon treatment for idiopathic hypereosinophilic syndrome. Am J Hematol. 1994;45:248–51.PubMedCrossRefGoogle Scholar
  14. 14.
    Butterfield JH, Gleich GJ. Interferon-alpha treatment of six patients with the idiopathic hypereosinophilic syndrome. Ann Intern Med. 1994;121:648–53.PubMedGoogle Scholar
  15. 15.
    Rothenberg ME, Kion AD, Roufosse FE, et al. Treatment of patients with Hypereosinophilic syndrome with mepolizumab. N Engl J Med. 2006;358:1215–28.CrossRefGoogle Scholar
  16. 16.
    Cools J, DeAngelo DJ, Gotlib J. 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;348:1201–14.PubMedCrossRefGoogle Scholar
  17. 17.
    Gleich GH, Leiferman KM, Pardanani A. Treatment of hypereosinophilic syndrome with imatinibmesilate. Lancet. 2002;359:1577–8.PubMedCrossRefGoogle Scholar
  18. 18.
    Archimbaud E, Guyotat D, Guillaume C, Godard J, Fiere D. Hypereosinophilic syndrome with multiple organ dysfunction treated by allogeneic bone marrow transplantation. Am J Hematol. 1988;27:302–3.PubMedCrossRefGoogle Scholar
  19. 19.
    Fukushima T, Kuriyama K, Ito H, et al. Successful bone marrow transplantation for idiopathic hypereosinophilic syndrome. Br J Haematol. 1995;90:213–5.PubMedCrossRefGoogle Scholar
  20. 20.
    Lefebvre C, Bletry O, Degoulet P, et al. Prognostic factors of hypereosinophilic syndrome. Study of 40 cases. Ann Med Interne (Paris). 1989;140:253–7.Google Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2012

Authors and Affiliations

  1. 1.Department of PediatricsKasturba Medical CollegeMangaloreIndia
  2. 2.Department of PediatricsKasturba Medical CollegeManipalIndia

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