Successful Treatment of Erdheim-Chester Disease with Multisystemic Involvement in a 4-year-old Child by Interferon-α and Corticosteroid
10.15264/cpho.2017.24.1.69
- Author:
Jung Won HWANG
1
;
Chang Hoon AN
;
Min Kyung LEE
;
Pyoung Han HWANG
Author Information
1. Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea. hwaph@jbnu.ac.kr
- Publication Type:Case Report
- Keywords:
Erdheim-Chester disease;
Non-Langerhans cell histiocytosis;
Interferon-α;
Corticosteroid
- MeSH:
Child;
Child, Preschool;
Erdheim-Chester Disease;
Histiocytosis;
Humans;
Male;
Middle Aged;
Mortality;
Prognosis
- From:Clinical Pediatric Hematology-Oncology
2017;24(1):69-74
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
