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KMID : 0387820170240010069
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Clinical Pediatric Hematology-Oncology
2017 Volume.24 No. 1 p.69 ~ p.74
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Successful Treatment of Erdheim-Chester Disease with Multisystemic Involvement in a 4-year-old Child by Interferon-α and Corticosteroid
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Hwang Jung-Won
An Chang-Hoon
Lee Min-Kyung
Hwang Pyoung-Han
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Abstract
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Erdheim-Chester disease (ECD) is a rare form of proliferative non-Langerhans cell histiocytosis that involves multiple organs and is associated with a high mortality. The prognosis of ECD is variable, and it mainly depends on the involved anatomic sites. The treatment modalities have not been standardized, but interferon-α (IFN-α) has been reported to be effective in the management of ECD. ECD usually affects middle aged individuals with a slight male predominance but is extremely rare in children. We present an uncommon case of a 4-year-boy diagnosed with ECD who was treated with IFN-α and corticosteroid. He remained disease-free for 3 years after the completion of treatment.
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KEYWORD
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Erdheim-Chester disease, Non-Langerhans cell histiocytosis, Interferon-α, Corticosteroid
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