儿童卡波西肉瘤：对致癌性人疱疹病毒8型的先天性或获得性免疫缺陷。
Kaposi Sarcoma of Childhood: Inborn or Acquired Immunodeficiency to Oncogenic HHV-8.
作者信息
Jackson Carolyn C, Dickson Mark A, Sadjadi Mahan, Gessain Antoine, Abel Laurent, Jouanguy Emmanuelle, Casanova Jean-Laurent
机构信息
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.
出版信息
Pediatr Blood Cancer. 2016 Mar;63(3):392-7. doi: 10.1002/pbc.25779. Epub 2015 Oct 15.
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.
摘要
卡波西肉瘤（KS）是一种由人类疱疹病毒8型（HHV-8）感染引起的内皮细胞恶性肿瘤。儿童KS的流行型和医源性型是由严重的后天性T细胞缺陷导致的。最近的研究表明，儿童经典型KS可能由罕见的单基因先天性免疫缺陷引起，涉及WAS、IFNGR1、STIM1和TNFRSF4基因的突变。儿童地方性KS的发病机制仍不清楚。我们综述了儿童KS的发病机制及其与遗传性和获得性免疫缺陷以及致癌性HHV-8的关系。
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