{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T01:24:17Z","timestamp":1771550657016,"version":"3.50.1"},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2006,9,8]],"date-time":"2006-09-08T00:00:00Z","timestamp":1157673600000},"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":[[2006,11]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>A total of 57 patients with Wiskott\u2013Aldrich syndrome (WAS) were studied after undergoing stem cell transplantation (SCT) in Japan between January 1985 and December 2004. Eleven patients received transplants from human leucocyte antigen (HLA)\u2010matched related donors, 10 from HLA\u2010mismatched related donors, 21 from unrelated bone marrow donors, and 15 from unrelated cord blood donors. Nine of the 57 patients rejected the initial graft. The overall 5\u2010year survival rate was 73\u00b77% and the 5\u2010year failure\u2010free survival rate was 65\u00b77% (failure was defined as rejection or death). The overall 5\u2010year survival rates for patients receiving bone marrow and cord blood from unrelated donors were both 80\u00b70%. Based on univariate analysis, the factors associated with poor survival were: transplantation from an HLA\u2010mismatched related donor, patient age of more than 5\u2003years at the time of transplantation, and a conditioning regimen other than busulfan and cyclophosphamide (BU\u2010CY) or busulfan, cyclophosphamide and antithymocyte globulin (BU\u2010CY\u2010ATG). In a multivariate analysis, a conditioning regimen other than BU\u2010CY and BU\u2010CY\u2010ATG was the only independent factor associated with transplantation failure. Given the improved outcome for WAS patients following transplantation from an unrelated donor, we conclude that patients with WAS should receive SCT as soon as possible after diagnosis.<\/jats:p>","DOI":"10.1111\/j.1365-2141.2006.06297.x","type":"journal-article","created":{"date-parts":[[2006,9,8]],"date-time":"2006-09-08T19:45:48Z","timestamp":1157744748000},"page":"362-366","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":83,"title":["Outcome in patients with Wiskott\u2013Aldrich syndrome following stem cell transplantation: an analysis of 57 patients in 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