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Treatment of CD40 ligand deficiency by hematopoietic stem cell transplantation: a survey of the European experience, 1993-2002

Gennery, A. R. (author)
Khawaja, K. (author)
Veys, P. (author)
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Bredius, R. G. (author)
Notarangelo, L. D. (author)
Mazzolari, E. (author)
Fischer, A. (author)
Landais, P. (author)
Cavazzana-Calvo, M. (author)
Friedrich, W. (author)
Fasth, Anders, 1945 (author)
Gothenburg University,Göteborgs universitet,Institutionen för kvinnors och barns hälsa, Avdelningen för pediatrik,Institute for the Health of Women and Children, Dept of Paediatrics
Wulffraat, N. M. (author)
Matthes-Martin, S. (author)
Bensoussan, D. (author)
Bordigoni, P. (author)
Lange, A. (author)
Pagliuca, A. (author)
Andolina, M. (author)
Cant, A. J. (author)
Davies, E. G. (author)
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 (creator_code:org_t)
2003-10-02
2004
English.
In: Blood. - : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 103:3, s. 1152-7
  • Journal article (peer-reviewed)
Abstract Subject headings
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  • CD40 ligand (CD40L) deficiency causes recurrent sinopulmonary infection, Pneumocystis carinii pneumonia, and Cryptosporidium parvum infection. Approximately 40% to 50% of patients survive to the third decade: long-term survival is unclear. Hematopoietic stem cell transplantation (HSCT) is curative. We present a retrospective analysis of 38 European patients undergoing HSCT for CD40L deficiency in 8 European countries between 1993 and 2002. Donor stem cell source included 14 HLA-identical siblings, 22 unrelated donors, and 2 phenotypically matched parental stem cells (12 T-cell depleted). Of the patients, 34 engrafted and 26 (68%) survived; 3 had autologous reconstitution, 22 (58%) were cured, and 1 engrafted but has poor T-cell immune reconstitution. There were 18 evaluated patients who responded to vaccination. Of the patients, 12 (32%) died from infection-related complications, with severe cryptosporidiosis in 6. Grades 2 to 4 graft-versus-host disease (GvHD) associated with infection occurred in 6 of 12 fatal cases. HSCT cured 58% of patients, 72% of those without hepatic disease. Early T-cell function following whole marrow HSCT may limit cryptosporidial disease, but survival was similar after T-cell-depleted HSCT. Preexisting lung damage was the most important adverse risk factor. Further studies will determine optimal timing and type of HSCT.

Keyword

Adolescent
Adult
CD40 Ligand/*genetics/*metabolism
Child
Child
Preschool
Data Collection
Europe
Genetic Diseases
X-Linked/genetics/immunology/*therapy
Graft Survival
Graft vs Host Disease/etiology
*Hematopoietic Stem Cell Transplantation/adverse effects
Humans
Hypergammaglobulinemia/genetics/immunology/*therapy
*Immunoglobulin M
Infant
Opportunistic Infections/etiology
Retrospective Studies

Publication and Content Type

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art (subject category)

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