Journal List > Korean J Hematol > v.40(3) > 1032628

Oh, Jang, Kang, Oh, and Chong: A Case of Acquired Glanzmann's Thrombasthenia

Abstract

Glanzmann's thrombasthenia (GT) is a rare inherited platelet disorder, which is characterized by a complete lack of platelet aggregation due to a deficiency or abnormality of the membrane glycoprotein IIb/IIIa complex. Anti-GPIIb/IIIa antibodies have also been identified to cause platelet dysfunction in patients with a normal platelet count, but this has only been rarely encountered. The condition is also known as acquired GT. Herein, we describe a patient with acquired GT and a history of Evans' syndrome, who presented with severe bleeding and platelet dysfunction, but with a normal platelet count and GP IIb/IIIa expression.

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Fig. 1.
Platelet aggregation test. (A) Platelet aggregation test with ristocetin shows near normal response. (B~D) Platelet aggregation test with collagen, ADP, thrombin shows complete absence of platelet aggregation.
kjh-40-183f1.tif
Fig. 2.
Flow-cytometric analysis of C D41a shows normal expression compared to control.
kjh-40-183f2.tif
Fig. 3.
Platelet aggregation test using control platelet and patient's plasma. (A~C) Results of platelet aggregation test show complete absence of platelet aggregation.
kjh-40-183f3.tif
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