A Case of Idiopathic Hypereosinophilic Syndrome |
Bin Cho, Jin Tack Kim, Joon Sung Lee, Kyoo Hong Cho |
Department of Pediatrics, Catholic University Medical College, Seoul, Korea |
특발성 과호산구성 증후군 1례 |
조빈, 김진택, 이준성, 조규홍 |
가톨릭대학교 의과대학 소아과학교실 |
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Abstract |
The idiopathic hypereosinophilic syndrome(HES) represents a heterogenous group of disorder characterized by prolonged eosinophilia of undetectable cause and multiorgan system dysfunction.
Bone marrow is the most frequentry involved organ, but the most severe clinicopathologic involvement is heart. The major cause of death in patients with the HES is cardiac dysfunction especially congestive heart failure resulted from endocardial fibrosis and restrictive cardiomyopathy.
We have experienced a case of HES with both cardiac and pulmonary involvements. The patient was an 18-month-old infant with poorly controlled lung abscess who complained of fever and productive cough. On the microscopic examination of pleural effusion, eosinophilic infiltration was noted. M-mode echocardiogram showed diffuse thickening of interventricular septum and left ventricular posterior wall. On the peripheral blood smear, prolonged eosinophilia was observed. We confirmed this case with marrow aspiration which showed eosinophilic hyperplasia. |
Key Words:
Idiopathic hypereosinophilic syndrome, Eosinophilia, HES |
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