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Pediatr Blood Cancer. 2016 Mar;63(3):392-7. doi: 10.1002/pbc.25779. Epub 2015 Oct 15.

Kaposi Sarcoma of Childhood: Inborn or Acquired Immunodeficiency to Oncogenic HHV-8.

Author information

1
St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York.
2
Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York.
3
Department of Medicine, Memorial Sloan Kettering Cancer Center, New York.
4
Department of Medicine, Weill Cornell Medical College, New York.
5
Unit of Epidemiology and Physiopathology of Oncogenic Viruses, Institut Pasteur, Paris, France.
6
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris, France.
7
Paris Descartes University, Imagine Institute, Paris, France.
8
Howard Hughes Medical Institute.
9
Pediatric Hematology-Immunology Unit, Necker Hospital for Sick Children, Paris, France.

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.

KEYWORDS:

HHV-8; Kaposi sarcoma; children; human herpes virus-8; pediatric

PMID:
26469702
PMCID:
PMC4984265
DOI:
10.1002/pbc.25779
[Indexed for MEDLINE]
Free PMC Article

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