%0 Journal Article
%T Modification of cellular and humoral immunity by somatically reverted T cells in X-linked lymphoproliferative syndrome type 1
%A Akihiro Hoshino
%A Hirokazu Kanegane
%A Hiroko Tanaka
%A Hisanori Fujino
%A Kay Tanita
%A Kenichi Chiba
%A Kenichi Yoshida
%A Koichi Ohshima
%A Kumiko Goi
%A Naonori Nishida
%A Satoru Miyano
%A Seiji Kojima
%A Seishi Ogawa
%A Takeyuki Kanzaki
%A Tomohiro Morio
%A Toshiaki Ono
%A Xi Yang
%A Yuichi Shiraishi
%A Yusuke Okuno
%J Journal of Allergy and Clinical Immunology
%D 2019
%R 10.1016/j.jaci.2018.07.044
%X Human SH2D1A mutations resulting in X-linked lymphoproliferative syndrome type 1 (XLP1)
are associated with a unique susceptibility to the EBV. This susceptibility is attributed
to impaired activation and cytotoxicity of CD8+ T cells by antigen-presenting B cells
but not by other antigen-presenting cells.1 Impaired function of CD4+ T cells contributes
to humoral immunodeficiency independent of EBV infection.2 In clinical situations,
fatal infectious mononucleosis (FIM)/hemophagocytic lymphohistiocytosis (HLH) have
high mortality rates, and hematopoietic stem cell transplantation (HSCT) is the only
curative therapy.
%U https://www.jacionline.org/article/S0091-6749(18)31288-0/abstract