Kaposi sarcoma of childhood: inborn or acquired immunodeficiency to oncogenic HHV‐8
CC Jackson, MA Dickson, M Sadjadi… - Pediatric blood & …, 2016 - Wiley Online Library
Pediatric blood & cancer, 2016•Wiley Online Library
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 …
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 …
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.
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