Successful Treatment of Erdheim-Chester Disease with Multisystemic Involvement in a 4-year-old Child by Interferon-α and Corticosteroid / 임상소아혈액종양
Clinical Pediatric Hematology-Oncology
;
: 69-74, 2017.
Article
in English
| WPRIM
| ID: wpr-788593
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Prognosis
/
Histiocytosis
/
Mortality
/
Erdheim-Chester Disease
Type of study:
Prognostic study
Limits:
Child
/
Child, preschool
/
Humans
/
Male
Language:
English
Journal:
Clinical Pediatric Hematology-Oncology
Year:
2017
Type:
Article
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