Kaposi Sarcoma of Childhood: Inborn or Acquired Immunodeficiency to Oncogenic HHV-8.
Pediatr Blood Cancer
; 63(3): 392-7, 2016 Mar.
Article
em En
| MEDLINE
| ID: mdl-26469702
ABSTRACT
Kaposi sarcoma (KS) is an endothelial malignancy caused by human herpes virus-8 (HHV-8) infection. The epidemic and iatrogenic forms of childhood KS result from a profound and acquired T cell deficiency. Recent studies have shown that classic KS of childhood can result from rare single-gene inborn errors of immunity, with mutations in WAS, IFNGR1, STIM1, and TNFRSF4. The pathogenesis of the endemic form of childhood KS has remained elusive. We review childhood KS pathogenesis and its relationship to inherited and acquired immunodeficiency to oncogenic HHV-8.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Sarcoma de Kaposi
/
Receptores de Interferon
/
Herpesvirus Humano 8
/
Proteína da Síndrome de Wiskott-Aldrich
/
Receptores OX40
/
Doenças Genéticas Inatas
/
Proteínas de Membrana
/
Proteínas de Neoplasias
Limite:
Child
/
Humans
Idioma:
En
Ano de publicação:
2016
Tipo de documento:
Article