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1.
Clinical Pediatric Hematology-Oncology ; : 69-74, 2017.
Artigo em Inglês | WPRIM | ID: wpr-197953

RESUMO

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.


Assuntos
Criança , Pré-Escolar , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Erdheim-Chester , Histiocitose , Mortalidade , Prognóstico
2.
Clinical Pediatric Hematology-Oncology ; : 69-74, 2017.
Artigo em Inglês | WPRIM | ID: wpr-788593

RESUMO

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.


Assuntos
Criança , Pré-Escolar , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Erdheim-Chester , Histiocitose , Mortalidade , Prognóstico
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