Kaposi sarcoma of childhood: Inborn or acquired immunodeficiency to oncogenic HHV-8 Journal Article


Authors: Jackson, C. C.; Dickson, M. A.; Sadjadi, M.; Gessain, A.; Abel, L.; Jouanguy, E.; Casanova, J. L.
Article Title: Kaposi sarcoma of childhood: Inborn or acquired immunodeficiency to oncogenic HHV-8
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. © 2016 Wiley Periodicals, Inc.
Keywords: cancer chemotherapy; gene mutation; disease course; mortality; review; antineoplastic agent; cancer diagnosis; morbidity; prevalence; childhood cancer; pediatric; carcinogenesis; correlation analysis; antivirus agent; antiretrovirus agent; highly active antiretroviral therapy; kaposi sarcoma; immunity; genetic disorder; immune deficiency; children; cd134 antigen; virus transmission; human herpesvirus 8; epidemic; antiviral therapy; gamma interferon receptor 1; herpes virus infection; wiskott aldrich syndrome protein; inheritance; hhv-8; cancer prognosis; human; priority journal; human herpes virus-8; stromal interaction molecule 1
Journal Title: Pediatric Blood and Cancer
Volume: 63
Issue: 3
ISSN: 1545-5009
Publisher: Wiley Periodicals, Inc  
Date Published: 2016-03-01
Start Page: 392
End Page: 397
Language: English
DOI: 10.1002/pbc.25779
PROVIDER: scopus
PUBMED: 26469702
PMCID: PMC4984265
DOI/URL:
Notes: Review -- Export Date: 3 March 2016 -- Source: Scopus
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  1. Mark Andrew Dickson
    169 Dickson
  2. Carolyn Chang Jackson
    11 Jackson