Diagnosis of Bernard-Soulier syndrome and Glanzmann's thrombasthenia with a monoclonal assay on whole blood

J Clin Invest. 1983 Feb;71(2):385-9. doi: 10.1172/jci110780.

Abstract

Two hereditary platelet disorders, Bernard-Soulier syndrome and Glanzmann's thrombasthenia, are characterized by selective deficiencies of platelet membrane glycoproteins. Murine monoclonal antibodies were developed against platelet membrane glycoprotein Ib and against the glycoprotein IIb/IIIa complex. A rapid whole blood assay for the deficiency of these glycoproteins was developed and used to study whole blood samples from six patients with Glanzmann's thrombasthenia and three patients with Bernard-Soulier syndrome. Patients with type I and type II Glanzmann's thrombasthenia were easily detectable with this assay. This permits the diagnosis of these disorders on 200 microliters of whole blood within 2 h of blood sampling.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Antibodies, Monoclonal / analysis*
  • Antibody Specificity
  • Autoradiography
  • Blood Platelet Disorders / diagnosis*
  • Blood Platelet Disorders / genetics
  • Blood Platelet Disorders / immunology
  • Cell Membrane / immunology
  • Glycoproteins / immunology
  • Humans
  • Mice
  • Mice, Inbred BALB C

Substances

  • Antibodies, Monoclonal
  • Glycoproteins