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 |