What condition is characterized by mucosal bleeding and prolonged bleeding time due to a defect in platelet aggregation?

Glanzmann's thrombasthenia is a rare inherited platelet disorder characterized by a defect in platelet aggregation, leading to mucosal bleeding and prolonged bleeding time. This condition is caused by a quantitative or qualitative defect in the glycoprotein IIb/IIIa complex, which is essential for platelet aggregation upon activation. When platelets cannot effectively aggregate, there is a significant impairment in the formation of a stable platelet plug, resulting in prolonged bleeding and easy bruising.

Patients with Glanzmann's thrombasthenia often present with symptoms such as epistaxis (nosebleeds), gum bleeding, and other mucosal hemorrhaging. The diagnosis can be confirmed through laboratory tests, which typically show normal platelet count but abnormal aggregation studies.

In distinguishing Glanzmann's thrombasthenia from other conditions, it’s helpful to note that von Willebrand disease is primarily characterized by a deficiency or dysfunction of von Willebrand factor, affecting both platelet function and coagulation, while the aspirin effect interferes with platelet function through inhibition of cyclooxygenase and thromboxane A2 production. Thrombocytopenic purpura involves a reduced platelet count, which can also lead to bleeding but is due to a different mechanism than the defective aggregation