Clin Pediatr Hematol Oncol.  2017 Apr;24(1):69-74. 10.15264/cpho.2017.24.1.69.

Successful Treatment of Erdheim-Chester Disease with Multisystemic Involvement in a 4-year-old Child by Interferon-α and Corticosteroid

• Hwang JW
• An CH
• Lee MK
• Hwang PH

• KMID: 2379353
• DOI: http://doi.org/10.15264/cpho.2017.24.1.69

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.

Keyword

Erdheim-Chester disease; Non-Langerhans cell histiocytosis; Interferon-α; Corticosteroid

MeSH Terms

Child*
Child, Preschool*
Erdheim-Chester Disease*
Histiocytosis
Humans
Male
Middle Aged
Mortality
Prognosis