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Analysis of Platelet Membrane Glycoprotein Ⅱb-Ⅲa complex in Whole Blood of Glanzmann's Thrombasthenia by Flow Cytometry

Journal of the Korean Pediatric Society 1994;37(11):1540-1547.
Published online November 15, 1994.
Analysis of Platelet Membrane Glycoprotein Ⅱb-Ⅲa complex in Whole Blood of Glanzmann's Thrombasthenia by Flow Cytometry
Byoung Geun Lee, Man Choon Kang, Jong Man Park, Pyung Han Hwang, Jung Soo Kim
Department of Pediatrics, School of Medicine, Chonbuk National University, Chonju, Korea
Glanzmann혈소판 무력증 환아의 전혈에서 Flow Cytometry를 이용한 혈소판막 당단백질 GP Ⅱb-Ⅲa 복합체의 분석
이병근, 강만춘, 박종만, 황평한, 김정수
전북대학교 의과대학 소아과학교실
Abstract
Glanzmann's thrompasthenia is a rare autosomal recessive hemorrhagic disorder characterized by prolonged bleeding time, and deficient or absent clot retraction in the presence of normal platelet count. The major underlying abnormality in this disease is grossly defective first-phase aggregation of platelet, which are unresponsive to ADP or other platelet agonists such as epinephrine, collagen, thrombin in any concentration. This disability is caused by a decrease of an absence of the platelet membrane glycoprotein Ⅱb-Ⅲa complex, a member of the integrin family of adhesive receptors involved in cell-cell and cell-matrix fibronectin, and vitronectin. On the development of surface labeling technique, a variety of biochemical techniques such as radiommunoassay, crossed immunoelectrophoresis and SDS-PAGE have been used to study the structure and the function of platelet membrane glycoproteins, and to detect the platelet functional defect. But all of these techniques demand a relatively large amount of homogeneous platelet population that requires manipulation through isolation and washing procedures before analysis. In order to eliminate such an intricate procedure, we have applied a method for analyzing platelet surface components in whole blood using monoclonal antibody and flow cytometry to recognize the absence or severe reduction of platelet membrane glycoproteinⅡb-Ⅲa complex. Platelet analysis by flow cytometry is a successful alternative rapid diagnostic technique for Glanzmann's thrombasthenia patients as well as for carriers of this disease. Flow cytometry technique provides a sensitive tool for investigating platelet functional defects caused by altered expression or deficiency of platelet surface proteins.
Key Words: Glanzmann's thrombasthenia, Flow cytometry


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