Glanzmann Thrombasthenia
Description
Impaired platelet aggregation from genetic deficiency of GpIIb/IIIa
Etiology
Autosomal recessive inheritance
Mechanism
↓
GpIIb/IIIa (a platelet integrin) → Defect in platelet plug formation
Symptoms and Signs
Bleeding from mucous membranes
Epistaxis, petechiae/purpura
Laboratory
↑
Bleeding time
Normal PT/PTT
Normal platelet count and morphology
Treatment
Platelet transfusions with HLA-matched and leukocyte depleted blood products
Supportive care (iron and folate supplementation)
Differential Diagnosis
Bernard-Soulier (↓ platelet count, ↑ bleeding time, and ↓ GpIb)
ITP (↓ platelet count, ↑ bleeding time, and anti-GpIIb/IIIa antibodies)
TTP (↓ platelet count, ↑ bleeding time, and defet/deficient ADAMTS 13)