Article
Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan.
Journal of clinical immunology - 1 Jan 2017
Ono Shintaro, Okano Tsubasa, Hoshino Akihiro, Yanagimachi Masakatsu, Hamamoto Kazuko, Nakazawa Yozo, Imamura Toshihiko, Onuma Masaei, Niizuma Hidetaka, Sasahara Yoji, Tsujimoto Hiroshi, Wada Taizo, Kunisaki Reiko, Takagi Masatoshi, Imai Kohsuke, Morio Tomohiro, Kanegane Hirokazu
Abstract excerpt
BACKGROUND: X-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency that is characterized by recurrent hemophagocytic lymphohistiocytosis (HLH) and splenomegaly and sometimes associated with refractory inflammatory bowel disease (IBD). Although hematopoietic stem cell transplantation (HSCT) is the only curative therapy, the outcomes of HSCT for XIAP deficiency remain unsatisfactory...
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