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

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.
AuthorsR R Montgomery, T J Kunicki, C Taves, D Pidard, M Corcoran
JournalThe Journal of clinical investigation (J Clin Invest) Vol. 71 Issue 2 Pg. 385-9 (Feb 1983) ISSN: 0021-9738 [Print] United States
PMID6822670 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Antibodies, Monoclonal
  • Glycoproteins
Topics
  • Animals
  • Antibodies, Monoclonal (analysis)
  • Antibody Specificity
  • Autoradiography
  • Blood Platelet Disorders (diagnosis, genetics, immunology)
  • Cell Membrane (immunology)
  • Glycoproteins (immunology)
  • Humans
  • Mice
  • Mice, Inbred BALB C

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