{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T15:18:43Z","timestamp":1787584723418,"version":"build-2736575974"},"reference-count":19,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2006,7,18]],"date-time":"2006-07-18T00:00:00Z","timestamp":1153180800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2006,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Major histocompatibility complex class II deficiency, a rare autosomal recessive primary immunodeficiency, is caused by the defective expression of human leucocyte antigen (HLA) class II molecules due to mutated <jats:italic>trans<\/jats:italic>\u2010acting elements of any one of four regulatory genes (<jats:italic>CIITA<\/jats:italic>, <jats:italic>RFXANK<\/jats:italic>, <jats:italic>RFX5<\/jats:italic>, <jats:italic>RFXAP<\/jats:italic>). The impaired CD4 T\u2010cell differentiation and antigen presentation in the periphery results in a severe defect of cellular and humoral response consistent with severe recurrent infections, leading to a poor prognosis. Currently, allogeneic haematopoietic stem cell transplantation (HSCT) is the only curative approach, but the overall cure rate is lower than in other immunodeficiencies. We report a single centre experience of 17 HSCTs with 15 HLA\u2010identical donors between 1981 and 2004. Eight patients survived, while the occurrence of acute graft\u2010<jats:italic>versus<\/jats:italic>\u2010host disease (GVHD) was 50%. This study aimed to identify potential risk factors for GVHD and outcome within pre\u2010HSCT complications related to the immunodeficiency. Five of seven patients with pre\u2010existing viral infections developed acute GVHD\u2003\u2265\u2003grade II, of whom four died. Two of seven patients without detectable pre\u2010existing viral infection developed GVHD\u2003\u2265\u2003grade II, and one died. The difference was significant (<jats:italic>P<\/jats:italic>\u2003&lt;\u20030\u00b705). A plausible link with other factors potentially associated with the development of GVHD could not be found. We suggest that the reduced survival after HLA\u2010identical HSCT may be caused by the high incidence of pre\u2010existing viral infections and associated with the onset of severe acute GVHD.<\/jats:p>","DOI":"10.1111\/j.1365-2141.2006.06213.x","type":"journal-article","created":{"date-parts":[[2006,7,18]],"date-time":"2006-07-18T09:20:12Z","timestamp":1153214412000},"page":"510-516","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":46,"title":["Human leucocyte antigen\u2010identical haematopoietic stem cell transplantation in major histocompatiblity complex class II immunodeficiency: reduced survival correlates with an increased incidence of acute graft\u2010<i>versus<\/i>\u2010host disease and pre\u2010existing viral infections"],"prefix":"10.1111","volume":"134","author":[{"given":"Raffaele","family":"Renella","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Capucine","family":"Picard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9n\u00e9dicte","family":"Neven","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Ouach\u00e9e\u2010Chardin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean\u2010Laurent","family":"Casanova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7oise 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