Idiopathic hypereosinophilic syndrome in children: 3 cases with review of literature

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.