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

作者: Carolyn C Jackson , Mark A Dickson , Mahan Sadjadi , Antoine Gessain , Laurent Abel

DOI: 10.1002/PBC.25779

关键词: SarcomaT cell deficiencyPathogenesisImmunologyMembrane proteinWiskott–Aldrich syndrome proteinAcquired immunodeficiency syndrome (AIDS)MedicineImmunityMalignancyVirology

摘要: 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 acquired T cell deficiency. Recent studies have shown that classic can rare single-gene inborn errors immunity, with mutations in WAS, IFNGR1, STIM1, TNFRSF4. pathogenesis the endemic form has remained elusive. We review its relationship to inherited immunodeficiency oncogenic HHV-8.

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