Glanzmann′s thrombasthenia: A case report and review

Abstract

Glanzmann′s thrombasthenia (GT) is a rare autosomal recessive disorder and characterized by a lack of platelet aggregation due to the absence of platelet glycoprotein (GP) Ilb and Illa. Usually, the disease leads to mild hemorrhage but sometimes bleeding is severe enough to be life-threatening. The site of bleeding in GT is clearly defined: purpura, epistaxis, gingival hemorrhage and menorrhagia are nearly constant features: gastrointestinal bleeding and hematuria are less common. In most cases, bleeding symptoms manifest rapidly after birth, even if GT is occasionally only diagnosed in later life. Diagnosis associates mucocutaneous bleeding with absent platelet aggregation in response to all physiologic stimuli, with normal platelet count and morphology. GT is more common in populations where marriage between blood relatives is common. Here, we present a case report of 24-year-old Indian male suffering from GT. A brief review of the relevant literature is also presented.

Keywords

• Bernard-Soulier syndrome (BSS)
• Congenital afibrinogenemia
• Epistaxis