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.
authors.affiliation: St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York.|Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York.
authors.forename: Carolyn C
authors.initials: CC
authors.lastname: Jackson
authors.affiliation: Department of Medicine, Memorial Sloan Kettering Cancer Center, New York.|Department of Medicine, Weill Cornell Medical College, New York.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Dickson
authors.affiliation: St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York.
authors.forename: Mahan
authors.initials: M
authors.lastname: Sadjadi
authors.affiliation: Unit of Epidemiology and Physiopathology of Oncogenic Viruses, Institut Pasteur, Paris, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Gessain
authors.affiliation: St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York.|Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris, France.|Paris Descartes University, Imagine Institute, Paris, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Abel
authors.affiliation: St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York.|Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris, France.|Paris Descartes University, Imagine Institute, Paris, France.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Jouanguy
authors.affiliation: St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York.|Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris, France.|Paris Descartes University, Imagine Institute, Paris, France.|Howard Hughes Medical Institute.|Pediatric Hematology-Immunology Unit, Necker Hospital for Sick Children, Paris, France.
authors.forename: Jean-Laurent
authors.initials: JL
authors.lastname: Casanova
mesh_terms: D002648:Child; D030342:Genetic Diseases, Inborn / Q000150:complications*; D019288:Herpesvirus 8, Human / Q000276:immunology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes; D008565:Membrane Proteins / Q000235:genetics*; D009363:Neoplasm Proteins / Q000235:genetics*; D017471:Receptors, Interferon / Q000235:genetics*; D053262:Receptors, OX40 / Q000235:genetics*; D012514:Sarcoma, Kaposi / Q000151:congenital* / Q000453:epidemiology / Q000276:immunology*; D000071737:Stromal Interaction Molecule 1; D051316:Wiskott-Aldrich Syndrome Protein / Q000235:genetics*; D000096743:Interferon gamma Receptor
chemical_list: D008565:Membrane Proteins; D009363:Neoplasm Proteins; D017471:Receptors, Interferon; D053262:Receptors, OX40; D000071737:Stromal Interaction Molecule 1; D051316:Wiskott-Aldrich Syndrome Protein; D000096743:Interferon gamma Receptor; C103530:STIM1 protein, human; C505716:TNFRSF4 protein, human; C495767:WAS protein, human
keywords: HHV-8; Kaposi sarcoma; children; human herpes virus-8; pediatric