All issues > Volume 34(2); 1991

Original Article

J Korean Pediatr Soc. 1991;34(2):287-291. Published online February 28, 1991.

A case of thrombasthenia.

Yong Bae Suh¹, Eun Kyoung Sohn¹, Yong Mook Choi¹, Chang Il Ahn¹

¹Department of Pediatrics, College of Medicine, Kyung Hee University, Seoul, Korea

Received: August 13, 1990;  Accepted: October 17, 1990.

Abstract

Glanzmann’s thrombasthenia is a congenital disorder of platelet function with missing or abnormal platelet plasma membrane glycoprotein Ilb/IIIa which function as receptors for fibrinogen and other high-molecular-weight adhesive proteins which are important for platelet aggregation. We have experienced a case of thrombasthenia in a 12 month old male whose chief complaints were easy bruising and frequent epistaxis. The beeding 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 aggregation response to ristocetin. Platelet adhesion was also decreased. The review of the literature was made briefly.

Keywords :Glanzmann's Thrombasthenia;Platelet aggregation test