title: Genotyping provides a reliable tool for the determination of the platelet antigen system HPA-1 in Glanzmann's thrombasthenia.
abstract: Glanzmann's thrombasthenia (GT) is an inherited disorder of platelet function, characterized by quantitative or qualitative defects of the platelet glycoprotein (GP) IIb/IIIa complex. Patients with GT may require repeated transfusions and therefore alloimmunization against platelet antigens could become of particular interest. As GPIIIa contains the most important platelet alloantigen system, HPA-1, its diminished expression in GT patients may impede serological determination of the HPA-1 allotype. By immunofluorescence consistent results were obtained in only two out of seven patients. The monoclonal antibody-specific immobilization of platelet antigen test allowed typing of six patients. DNA analysis was feasible in all cases. All seven patients were identified to be homozygous HPA-1a. Thus, provided a normal HPA-1 DNA region, its analysis can serve as a reliable tool for HPA-1 typing in GT even if serological methods fail.
authors.affiliation: Institute for Blood Group Serology, University of Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Pober
authors.forename: P A
authors.initials: PA
authors.lastname: Kyrle
authors.forename: S
authors.initials: S
authors.lastname: Panzer
mesh_terms: D016824:Antigens, Human Platelet / Q000097:blood* / Q000235:genetics; D001483:Base Sequence; D001792:Blood Platelets / Q000276:immunology*; D004247:DNA / Q000032:analysis; D005838:Genotype; D006801:Humans; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction; D013915:Thrombasthenia / Q000276:immunology*
chemical_list: D016824:Antigens, Human Platelet; D004247:DNA