title: Hematopoietic Stem Cell Transplant for Immune Deficiency and Immune Dysregulation Disorders.
abstract: Primary immunodeficiency disorders were among the first diseases in which hematopoietic stem cell transplant (HSCT) was attempted. Initial attempts at HSCT were discouraging and fraught with complications, but with increased knowledge and sophistication of HLA typing and donor matching, development of improved transplant conditioning regimens, and advances in prophylaxis and treatment of graft-versus-host disease, there has been a marked improvement in outcomes. This improvement has allowed an ever-growing number of different immunodeficiency and immune dysregulation disorders to be treated by HSCT. This article provides an overview of the approach to HSCT in these disorders.
authors.affiliation: Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA 98105, USA; University of Washington School of Medicine, 1959 NE Pacific St, Seattle, WA 98195, USA; Seattle Children's Research Institute, 1900 9th Avenue, JMB-7, Seattle, WA 98101-1304, USA.
authors.forename: David
authors.initials: D
authors.lastname: Hagin
authors.affiliation: Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA 98105, USA; University of Washington School of Medicine, 1959 NE Pacific St, Seattle, WA 98195, USA; Fred Hutchinson Cancer Research Center, 1100 Fairview Avenue North, Suite D3-100, Seattle, WA 98109, USA.
authors.forename: Lauri
authors.initials: L
authors.lastname: Burroughs
authors.affiliation: Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA 98105, USA; University of Washington School of Medicine, 1959 NE Pacific St, Seattle, WA 98195, USA; Seattle Children's Research Institute, 1900 9th Avenue, JMB-7, Seattle, WA 98101-1304, USA. Electronic address: troy.torgerson@seattlechildrens.org.
authors.forename: Troy R
authors.initials: TR
authors.lastname: Torgerson
mesh_terms: D046148:Donor Selection; D006084:Graft Rejection / Q000276:immunology; D006086:Graft vs Host Disease / Q000209:etiology / Q000517:prevention & control; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects / Q000379:methods; D006412:Hematopoietic Stem Cells / Q000166:cytology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D018183:Transplantation Chimera; D019172:Transplantation Conditioning / Q000379:methods; D016896:Treatment Outcome
keywords: BMT; Conditioning; GVHD; GVT; HSCT; PIDD; Rejection