TY - JOUR
T1 - Idiopathic hypereosinophilic syndrome in children
T2 - 3 cases with review of literature
AU - Kanthila, Jayashree
AU - Bhaskaranand, Nalini
PY - 2013/2/1
Y1 - 2013/2/1
N2 - 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.
AB - 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.
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U2 - 10.1007/s12098-012-0821-x
DO - 10.1007/s12098-012-0821-x
M3 - Review article
C2 - 22740191
AN - SCOPUS:84877046794
SN - 0972-9607
VL - 80
SP - 124
EP - 127
JO - Indian Journal of Practical Pediatrics
JF - Indian Journal of Practical Pediatrics
IS - 2
ER -