About: Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan

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An Entity of Type : schema:ScholarlyArticle, within Data Space : covidontheweb.inria.fr associated with source document(s)

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type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan
Creator	Kanegane, Hirokazu Hamamoto, Kazuko Hoshino, Akihiro Imai, Kohsuke Imamura, Toshihiko Kunisaki, Reiko Morio, Tomohiro Nakazawa, Yozo Niizuma, Hidetaka Okano, Tsubasa Ono, Shintaro Onuma, Masaei Sasahara, Yoji Takagi, Masatoshi Tsujimoto, Hiroshi Wada, Taizo Yanagimachi, Masakatsu
Source	PMC
abstract	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 compared with those for SLAM-associated protein deficiency and familial HLH. AIM: To investigate the outcomes and adverse events of HSCT for patients with XIAP deficiency, a national survey was conducted. METHODS: A spreadsheet questionnaire was sent to physicians who had provided HSCT treatment for patients with XIAP deficiency in Japan. RESULTS: Up to the end of September 2016, 10 patients with XIAP deficiency had undergone HSCT in Japan, 9 of whom (90%) had survived. All surviving patients had received a fludarabine-based reduced intensity conditioning (RIC) regimen. Although 5 patients developed post-HSCT HLH, 4 of them survived after etoposide administration. In addition, the IBD associated with XIAP deficiency improved remarkably after HSCT in all affected cases. CONCLUSION: The RIC regimen and HLH control might be important factors for successful HSCT outcomes, with improved IBD, in patients with XIAP deficiency. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1007/s10875-016-0348-4) contains supplementary material, which is available to authorized users.
has issue date	2016-11-04(xsd:dateTime)
bibo:doi	10.1007/s10875-016-0348-4
bibo:pmid	27815752
has license	no-cc
sha1sum (hex)	57b13e969089ce14fba5884fc2c34b0c9a09edf2
schema:url	https://doi.org/10.1007/s10875-016-0348-4
resource representing a document's title	Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan
has PubMed Central identifier	PMC7101905
has PubMed identifier	27815752
schema:publication	J Clin Immunol
resource representing a document's body	covid:57b13e969089ce14fba5884fc2c34b0c9a09edf2#body_text
is schema:about of	named entity 'apoptosis' named entity 'XIAP' named entity 'OUTCOMES' named entity 'RECURRENT HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS' named entity 'PHYSICIANS' named entity 'compared' named entity 'conducted' named entity 'X-linked' named entity 'XIAP' named entity 'adverse events' named entity 'HSCT' named entity 'XIAP' named entity 'XIAP' named entity 'hematopoietic stem cell transplantation' named entity 'immunodeficiency' named entity 'Japan' named entity 'Hematopoietic Stem Cell Transplantation' named entity 'IBD' named entity 'IBD' named entity 'SAP' named entity 'HLH' named entity 'XIAP' named entity 'chimerism' named entity 'conditioning regimens' named entity 'RIC' named entity 'XIAP' named entity 'XIAP' named entity 'TBI' named entity 'tacrolimus' named entity 'HSCT' named entity 'chimerism' named entity 'nucleotide' named entity 'fludarabine' named entity 'conditioning regimen' named entity 'IBD' named entity 'HLH' named entity 'dexamethasone' named entity 'alemtuzumab' named entity 'total body irradiation' named entity 'inhibitor of apoptosis' named entity 'HSCT' named entity 'virus' named entity 'etoposide' named entity 'HSCT' named entity 'virus' named entity 'HSCT' named entity 'IBD' named entity 'autoinflammatory' named entity 'protein' named entity 'XIAP' named entity 'prognosis' named entity 'graft source' named entity 'HLH' named entity 'ulcers' named entity 'IBD' named entity 'ATG' named entity 'TBI' named entity 'bone marrow' named entity 'GVHD' named entity 'dysgammaglobulinemia' named entity 'HSCT' named entity 'SAP' named entity 'colon' named entity 'virus infection' named entity 'cord blood'

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