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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
5
|
| pubmed:dateCreated |
1996-11-6
|
| pubmed:abstractText |
Seven male patients in the David T Purtilo International X-linked Lymphoproliferative Disease (XLP) Registry have undergone allogeneic hematopoietic stem cell transplantation (HSCT). All patients received HSCT from HLA-identical donors: sibling BM, five; unrelated BM, one; and sibling umbilical cord blood, one. Ages at time of HSCT ranged from 5 to 30 years. Pre-HSCT clinical course varied, but four boys had a significant history of chronic and/or serious infections. Conditioning regimens varied: TBI containing regimens, four, chemotherapy only, three. All patients engrafted. Six developed grade I-II acute GVHD but no chronic GVHD. Four are alive and well with normal immune function greater than 3 years following HSCT. Three died within 100 days: disseminated adenovirus, one; polymicrobial sepsis, one; and multiple organ system failure and bleeding diathesis, one. No EBV-associated post-transplant complications were observed, even though all donors except the umbilical cord blood were EBV-seropositive. Unsuccessful HSCT was associated with age at HSCT (> 15 years), TBI-containing regimen and significant history for pre-HSCT infections. These results provide evidence that HSCT performed during childhood with HLA-identical sibling donors, regardless of EBV serostatus, offers the only curative therapy for XLP.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
May
|
| pubmed:issn |
0268-3369
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:volume |
17
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
741-4
|
| pubmed:dateRevised |
2010-11-18
|
| pubmed:meshHeading |
pubmed-meshheading:8733691-Adolescent,
pubmed-meshheading:8733691-Adult,
pubmed-meshheading:8733691-Antibodies, Viral,
pubmed-meshheading:8733691-Child,
pubmed-meshheading:8733691-Child, Preschool,
pubmed-meshheading:8733691-Female,
pubmed-meshheading:8733691-Fetal Blood,
pubmed-meshheading:8733691-Genetic Linkage,
pubmed-meshheading:8733691-Graft Survival,
pubmed-meshheading:8733691-Graft vs Host Disease,
pubmed-meshheading:8733691-HLA Antigens,
pubmed-meshheading:8733691-Hematopoietic Stem Cell Transplantation,
pubmed-meshheading:8733691-Herpesvirus 4, Human,
pubmed-meshheading:8733691-Humans,
pubmed-meshheading:8733691-Living Donors,
pubmed-meshheading:8733691-Lymphoproliferative Disorders,
pubmed-meshheading:8733691-Male,
pubmed-meshheading:8733691-Registries,
pubmed-meshheading:8733691-Transplantation, Homologous,
pubmed-meshheading:8733691-Transplantation Conditioning,
pubmed-meshheading:8733691-X Chromosome
|
| pubmed:year |
1996
|
| pubmed:articleTitle |
Cure of X-linked lymphoproliferative disease (XLP) with allogeneic hematopoietic stem cell transplantation (HSCT): report from the XLP registry.
|
| pubmed:affiliation |
Department of Pathology@Microbiology, University of Nebraska Medical Center, Omaha 68198-5660, USA.
|
| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|