Genotyping provides a reliable tool for the determination of the platelet antigen system HPA‐1 in Glanzmann's thrombasthenia

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Abstract
Summary. 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 immunofluores‐cence 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‐la. 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.