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

Br J Haematol. 1993 Sep;85(1):112-5. doi: 10.1111/j.1365-2141.1993.tb08653.x.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antigens, Human Platelet / blood*
  • Antigens, Human Platelet / genetics
  • Base Sequence
  • Blood Platelets / immunology*
  • DNA / analysis
  • Genotype
  • Humans
  • Molecular Sequence Data
  • Polymerase Chain Reaction
  • Thrombasthenia / immunology*

Substances

  • Antigens, Human Platelet
  • DNA