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

作者: R R Montgomery , T J Kunicki , C Taves , D Pidard , M Corcoran

DOI: 10.1172/JCI110780

关键词: Bernard–Soulier syndromePlatelet disorderImmunologyThrombastheniaPlatelet membrane glycoproteinBlood Platelet DisordersWhole bloodMedicineBlood samplingGlanzmann's thrombasthenia

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

参考文章(15)
RR Montgomery, WE Hathaway, J Johnson, L Jacobson, W Muntean, A variant of von Willebrand's disease with abnormal expression of factor VIII procoagulant activity Blood. ,vol. 60, pp. 201- 207 ,(1982) , 10.1182/BLOOD.V60.1.201.201
L.Michael Glode, Robert R. Montgomery, Christopher G. Smith, David B. Link, Use of monoclonal antibody to increase sensitivity and specificity in quantitative immunodiffusion assays. Journal of Immunological Methods. ,vol. 48, pp. 13- 22 ,(1982) , 10.1016/0022-1759(82)90205-8
James N. George, Terry A. Reimann, Joel L. Moake, Rebecca K. Morgan, Phillip L. Cimo, David A. Sears, Bernard-Soulier Disease: a Study of Four Patients and their Parents British Journal of Haematology. ,vol. 48, pp. 459- 467 ,(1981) , 10.1111/J.1365-2141.1981.TB02738.X
T J Kunicki, M M Johnson, R H Aster, Absence of the platelet receptor for drug-dependent antibodies in the Bernard-Soulier syndrome. Journal of Clinical Investigation. ,vol. 62, pp. 716- 719 ,(1978) , 10.1172/JCI109181
A. T. Nurden, J. P. Caen, An abnormal platelet glycoprotein pattern in three cases of Glanzmann's thrombasthenia. British Journal of Haematology. ,vol. 28, pp. 253- 260 ,(1974) , 10.1111/J.1365-2141.1974.TB06660.X
Rodger P. McEver, Nancy Lewis Baenziger, Philip W. Majerus, Isolation and Quantitation of the Platelet Membrane Glycoprotein Deficient in Thrombasthenia Using a Monoclonal Hybridoma Antibody Journal of Clinical Investigation. ,vol. 66, pp. 1311- 1318 ,(1980) , 10.1172/JCI109983
P J Newman, R A Kahn, A Hines, Detection and characterization of monoclonal antibodies to platelet membrane proteins. Journal of Cell Biology. ,vol. 90, pp. 249- 253 ,(1981) , 10.1083/JCB.90.1.249
DAVID R. PHILLIPS, C. S. P. JENKINS, E. F. LÜSCHER, MARIE-JOSE LARRIEU, Molecular differences of exposed surface proteins on thrombasthenic platelet plasma membranes Nature. ,vol. 257, pp. 599- 600 ,(1975) , 10.1038/257599A0
A. J. McMichael, N. A. Rust, J. R. Pilch, R. Sochynsky, J. Morton, D. Y. Mason, C. Ruan, G. Tobelem, J. Caen, Monoclonal antibody to human platelet glycoprotein I. I. Immunological studies. British Journal of Haematology. ,vol. 49, pp. 501- 509 ,(1981) , 10.1111/J.1365-2141.1981.TB07258.X