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A Case of Type Ⅰ Glanzmann's Thrombasthenia Diagnosed by Flow Cytometry

Journal of the Korean Pediatric Society 1999;42(1):133-137.
Published online January 15, 1999.
A Case of Type Ⅰ Glanzmann's Thrombasthenia Diagnosed by Flow Cytometry
Mun Su Lee1, Choong Ho Shin1, Kyu Young Kim1, Dong Woo Son1, Hwa Ryung Chung2, Do Hyun Kim1
1Department of Pediatrics, Eul Ji Medical Center, Seoul, Korea
2Department of Clinical Pathology, Eul Ji Medical Center, Seoul, Korea
Flow Cytometry로 진단된 혈소판 무력증 제1형 1례
이문수1, 신충호1, 김규영1, 손동우1, 정화령2, 김도현1
1노원을지병원 소아과
2노원을지병원 임상병리과
Abstract
Glanzmann's thrombasthenia is a rare autosomal recessive hemorrhagic disorder of platelet function with missing or abnormal platelet plasma membrane glycoprotein Ⅱb-Ⅲa, which functions as a receptor for fibrinogen. We have experienced a case of thrombasthenia in a 6-year-old female whose chief complaints were easy bruising, frequent epistaxis, arthralgia and swelling of the right ankle joint. Bleeding time was prolonged in the presence of normal platelet levels and the platelet aggregation test showed lack of aggregation after exposure to ADP, epinephrine and collagen, but showed an aggregation response to ristocetin. 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 an effective tool for investigating platelet function defects caused by altered expression or deficiency of platelet surface proteins.
Key Words: Glanzmann's thrombasthenia, Flow cytometry


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