GLANZMANN'S THROMBASTHENIA

• Biochemical aspects:
  • Platelets inpatients with this disorder have decreased amounts of membrane glycoproteins IIb and IIIa. It has also been demonstrated that the level of platelet- specific antigen PLA1 is decreased in these patients. This antigen marker is associated with membrane glycoprotein IIIa.
  • The relationship between membrane glycoproteins and functional abnormalities of aggregation and clot retraction in Glanzmann's thrombasthenia is currently unknown.
• Pathological Basis:
  • Mode of inheritance: autosomal recessive.
  • The differential dignoses include: storage pool disease, von Willebrand disease.
• Treatment:
  • Judicious use of platelet concentrates (platelet antibodies may develop).
  • Avoidance of aspirin-containing compounds.