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. 1978 May;61(5):1225–1231. doi: 10.1172/JCI109038

Deletion of the Platelet-Specific Alloantigen PlA1 from Platelets in Glanzmann's Thrombasthenia

Thomas J Kunicki 1,2,3, Richard H Aster 1,2,3
PMCID: PMC372643  PMID: 566280

Abstract

Expression of a Platelet-specific alloantigen (PlA1) was studied in five unrelated patients with Glanzmann's thrombasthenia using immunologic techniques based on release of 51Cr from tagged platelets by PlA1-specific antibody. Less than 1% of the normal quantity of PlA1 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 51Cr as well as normal PlA1-positive platelets when exposed to anti-PlA1. 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 IIbα 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 IIbα and III were found in platelets from normal subjects negative for PlA1.

These observations are consistent with the possibility that the PlA1 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 PlA1 antigen can be measured with considerable precision and may provide a marker useful for the diagnosis and study of Glanzmann's disease.

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Selected References

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