| The integrin receptor glycoprotein (GP) IIb/IIIa, expressed on the surface of platelets, mediates the formation of platelet aggregates at the site of vessel injury. Glanzmann thrombasthenia is an autosomal recessive bleeding disorder characterized by the absence of platelet aggregation due to the absence or aberrant expression of GPIIb/IIIa. More than 30 genetic defects in the genes that encode GPIIb or GPIIIa have been identified that are responsible for Glanzmann thrombasthenia. A 20-year-old woman from the Hunan Province of the People's Republic of China was diagnosed as having Glanzmann thrombasthenia. The patient's platelets had a decreased level of platelet fibrinogen, but not as severe as in other Glanzmann thrombasthenia patients. As judged by monoclonal antibody (mAb) binding studies, surface GPIIb/IIIa (... |
