Korean J Hematol.  2005 Sep;40(3):183-187. 10.5045/kjh.2005.40.3.183.

A Case of Acquired Glanzmann's Thrombasthenia

• Oh SY
• Jang MJ
• Kang MS
• Oh D
• Chong SY

• KMID: 1610813
• DOI: http://doi.org/10.5045/kjh.2005.40.3.183

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.

Keyword

Acquired thrombasthenia; Evans' syndrome; Platelet aggregation

MeSH Terms

Antibodies
Blood Platelets
Hemorrhage
Humans
Membrane Glycoproteins
Platelet Aggregation
Platelet Count
Thrombasthenia*
Antibodies
Membrane Glycoproteins

Figure

• 

  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.

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  Fig. 2. Flow-cytometric analysis of C D41a shows normal expression compared to control.

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  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.

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