title: Fatal graft versus host disease after platelet transfusions in a child with purine nucleoside phosphorylase deficiency.
abstract: Fatal graft versus host disease (GVHD) developed in a child with purine nucleoside phosphorylase (PNP) deficiency following an unirradiated platelet transfusion. IV treatment with an anti-T-cell monoclonal antibody (CD7) led to a transient improvement of his GVHD (Grade IV) but did not prevent the fatal outcome. This report emphasizes the need for blood products to be irradiated when cell mediated immunodeficiency is suspected, even in patients with residual immunocompetence.
authors.affiliation: Department of Immunology, Institute of Child Health, London, United Kingdom.
authors.forename: S
authors.initials: S
authors.lastname: Strobel
authors.forename: G
authors.initials: G
authors.lastname: Morgan
authors.forename: A H
authors.initials: AH
authors.lastname: Simmonds
authors.forename: R J
authors.initials: RJ
authors.lastname: Levinsky
mesh_terms: D001803:Blood Transfusion; D002675:Child, Preschool; D006580:Genetic Carrier Screening; D006086:Graft vs Host Disease / Q000201:enzymology* / Q000473:pathology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000201:enzymology* / Q000235:genetics; D008297:Male; D010430:Pentosyltransferases / Q000172:deficiency*; D017713:Platelet Transfusion; D011683:Purine-Nucleoside Phosphorylase / Q000172:deficiency*; D012867:Skin / Q000473:pathology
chemical_list: D010430:Pentosyltransferases; D011683:Purine-Nucleoside Phosphorylase