Jayashree Kanthila1, Nalini Bhaskaranand. 1. Department of Pediatrics, Kasturba Medical College, A-14, A Block, KMC Staff Quarters, Light House Hill Road, Mangalore, 575003, India. jayashreedoc@gmail.com
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/mm(3)) 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.
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/mm(3)) 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.
Authors: Jan Cools; Daniel J DeAngelo; Jason Gotlib; Elizabeth H Stover; Robert D Legare; Jorges Cortes; Jeffrey Kutok; Jennifer Clark; Ilene Galinsky; James D Griffin; Nicholas C P Cross; Ayalew Tefferi; James Malone; Rafeul Alam; Stanley L Schrier; Janet Schmid; Michal Rose; Peter Vandenberghe; Gregor Verhoef; Marc Boogaerts; Iwona Wlodarska; Hagop Kantarjian; Peter Marynen; Steven E Coutre; Richard Stone; D Gary Gilliland Journal: N Engl J Med Date: 2003-03-27 Impact factor: 91.245