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Deletion of the platelet-specific alloantigen PlA1 from platelets in Glanzmann's thrombasthenia. J Clin Invest 1978 May;61(5):1225-31 PMID: 566280 PMCID: PMC372643

Pubmed ID

566280

Abstract

Expression of a Platelet-specific alloantigen (Pl(A1)) was studied in five unrelated patients with Glanzmann's thrombasthenia using immunologic techniques based on release of (51)Cr from tagged platelets by Pl(A1)-specific antibody. Less than 1% of the normal quantity of Pl(A1) could be detected on platelets of patients 1, 2, and 3; platelets from patients 4 and 5 contained 22 and 12% of normal levels, respectively. After treatment with bromelain, platelets from patients 4 and 5, but not those from patients 1, 2, and 3, released (51)Cr as well as normal Pl(A1)-positive platelets when exposed to anti-Pl(A1). Platelets from each of the five patients reacted normally with drug-dependent antibodies and with autoantibodies specific for platelets. Polyacrylamide gel electrophoresis of thrombasthenic platelets showed marked deficiencies of glycoproteins IIbalpha and III (P < 0.0005), confirming recent reports of others. Deficiency of the two proteins as determined by gel scanning was more pronounced in patients 1, 2, and 3 than in patients 4 and 5. Normal levels of glycoproteins IIbalpha and III were found in platelets from normal subjects negative for Pl(A1). These observations are consistent with the possibility that the Pl(A1) antigen is located on one or both of the glycoproteins lacking in Glanzmann's thrombasthenia, although other explanations are possible. They further suggest that patients with thrombasthenia may be heterogeneous in respect to the degree to which these glycoproteins are deleted. The Pl(A1) antigen can be measured with considerable precision and may provide a marker useful for the diagnosis and study of Glanzmann's disease.

Author List

Kunicki TJ, Aster RH

Author

Richard H. Aster MD Professor in the Medicine department at Medical College of Wisconsin




Scopus

2-s2.0-0017874970   50 Citations

MESH terms used to index this publication - Major topics in bold

Blood Platelets
Bromelains
Glycoproteins
Humans
Isoantigens
Membrane Proteins
Purpura, Thrombocytopenic
jenkins-FCD Prod-353 9ccd8489072cb19f5b9f808bb23ed672c582f41e