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A Case of Glanzmann's Thromboasthenia.

Journal of the Korean Pediatric Society 1987;30(2):223-228.
Published online February 28, 1987.
A Case of Glanzmann's Thromboasthenia.
Hee Kyung Park, Kyung Hee Kim, Sang Man Shin, Sang Jhoo Lee
Dept, of Pediatrics, College of Medicine, Soon Cheon Hyang University
혈소판 무력증 1례
박희경, 김경희, 신상만, 이상주
순천향대학 의과대학 소아과학교실
Abstract
Glanzmann’s Thromboasthenia is a rare autosomal recessive hemorrhagic disorder characterized by chronic nonthrombocytopenic purpura, a prolonged bleeding time, and deficient or absent clot retraction. The major underlying abnormality is defective first phase aggregation of platelets, which are unresponsive to ADP in any concentration. The authors experienced one case of Glanzmann’s Thromboasthenia in a 8 year 10 month old female, who had frequent epistaxis and gingival bleeding. Platelets of patient showed complete absence of aggregation in response to ADP, collagen and epinephrine. The review of the literature was made briefly.
Key Words: Thromboasthenia, Platelet aggregation.


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