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. 1983 Feb;71(2):385–389. doi: 10.1172/JCI110780

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

R R Montgomery, T J Kunicki, C Taves, D Pidard, M Corcoran
PMCID: PMC436878  PMID: 6822670

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.

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Selected References

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  1. George J. N., Reimann T. A., Moake J. L., Morgan R. K., Cimo P. L., Sears D. A. Bernard-Soulier disease: a study of four patients and their parents. Br J Haematol. 1981 Jul;48(3):459–467. doi: 10.1111/j.1365-2141.1981.tb02738.x. [DOI] [PubMed] [Google Scholar]
  2. Glode L. M., Montgomery R. R., Smith C. G., Link D. B. Use of monoclonal antibody to increase sensitivity and specificity in quantitative immunodiffusion assays. J Immunol Methods. 1982;48(1):13–22. doi: 10.1016/0022-1759(82)90205-8. [DOI] [PubMed] [Google Scholar]
  3. Jamieson G. A., Okumura T., Fishback B., Johnson M. M., Egan J. J., Weiss H. J. Platelet membrane glycoproteins in thrombasthenia, Bernard-Soulier syndrome, and storage pool disease. J Lab Clin Med. 1979 Apr;93(4):652–660. [PubMed] [Google Scholar]
  4. Katzmann J. A., Mujwid D. K., Miller R. S., Fass D. N. Monoclonal antibodies to von Willebrand's factor: reactivity with porcine and human antigens. Blood. 1981 Sep;58(3):530–536. [PubMed] [Google Scholar]
  5. Kunicki T. J., Johnson M. M., Aster R. H. Absence of the platelet receptor for drug-dependent antibodies in the Bernard-Soulier syndrome. J Clin Invest. 1978 Sep;62(3):716–719. doi: 10.1172/JCI109181. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Kunicki T. J., Nurden A. T., Pidard D., Russell N. R., Caen J. P. Characterization of human platelet glycoprotein antigens giving rise to individual immunoprecipitates in crossed-immunoelectrophoresis. Blood. 1981 Dec;58(6):1190–1197. [PubMed] [Google Scholar]
  7. Kunicki T. J., Pidard D., Rosa J. P., Nurden A. T. The formation of Ca++-dependent complexes of platelet membrane glycoproteins IIb and IIIa in solution as determined by crossed immunoelectrophoresis. Blood. 1981 Aug;58(2):268–278. [PubMed] [Google Scholar]
  8. McEver R. P., Baenziger N. L., Majerus P. W. Isolation and quantitation of the platelet membrane glycoprotein deficient in thrombasthenia using a monoclonal hybridoma antibody. J Clin Invest. 1980 Dec;66(6):1311–1318. doi: 10.1172/JCI109983. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. McMichael A. J., Rust N. A., Pilch J. R., Sochynsky R., Morton J., Mason D. Y., Ruan C., Tobelem G., Caen J. Monoclonal antibody to human platelet glycoprotein I. I. Immunological studies. Br J Haematol. 1981 Dec;49(4):501–509. doi: 10.1111/j.1365-2141.1981.tb07258.x. [DOI] [PubMed] [Google Scholar]
  10. Montgomery R. R., Hathaway W. E., Johnson J., Jacobson L., Muntean W. A variant of von Willebrand's disease with abnormal expression of factor VIII procoagulant activity. Blood. 1982 Jul;60(1):201–207. [PubMed] [Google Scholar]
  11. Newman P. J., Kahn R. A., Hines A. Detection and characterization of monoclonal antibodies to platelet membrane proteins. J Cell Biol. 1981 Jul;90(1):249–253. doi: 10.1083/jcb.90.1.249. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Nurden A. T., Caen J. P. An abnormal platelet glycoprotein pattern in three cases of Glanzmann's thrombasthenia. Br J Haematol. 1974 Oct;28(2):253–260. doi: 10.1111/j.1365-2141.1974.tb06660.x. [DOI] [PubMed] [Google Scholar]
  13. Nurden A. T., Caen J. P. The different glycoprotein abnormalities in thrombasthenic and Bernard-Soulier platelets. Semin Hematol. 1979 Jul;16(3):234–250. [PubMed] [Google Scholar]

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