title: Stem cell transplantation for primary immunodeficiency disease: experience of a singapore hospital.
abstract: We retrospectively analyzed the outcomes of hematopoietic stem cell transplantation in 7 patients with primary immunodeficiency diseases treated at the National University Hospital, Singapore, over the period from December 1996 to January 2010. The primary immunodeficiency diseases managed were X-linked hyperimmunoglobulin M syndrome (n = 3), severe combined immunodeficiency (n = 1), leukocyte adhesion deficiency type 1 (n = 1), chronic granulomatous disease (n = 1), and Wiskott-Aldrich syndrome (n = 1). The age of the patients ranged from 5 months to 17 years. Conditioning regimen depended on the type of immunodeficiency, whereas supportive treatment was tailored for differing pretransplant conditions. Eight stem cell transplantations were performed for 7 patients. Donors were HLA-matched sibling donors for 2 patients and unrelated donors for the rest. At the median follow-up of 8.6 years (range 2.2-15.0 years) as of December 2011, 6 patients were alive and cured of their primary diseases.
authors.affiliation: Department of Paediatrics, University Children's Medical Institute, National University Hospital, Singapore.
authors.forename: Alison Joanne
authors.initials: AJ
authors.lastname: Lee
authors.forename: Jethro
authors.initials: J
authors.lastname: Wu
authors.forename: Mariflor Sarmiento
authors.initials: MS
authors.lastname: Villegas
authors.forename: Lynette Pei-Chi
authors.initials: LP
authors.lastname: Shek
authors.forename: Bee-Wah
authors.initials: BW
authors.lastname: Lee
authors.forename: Poh-Lin
authors.initials: PL
authors.lastname: Tan