Glanzmann's thrombasthenia is a rare hereditary defect in platelet aggregation, associated with defects in the genes for platelet glycoprotein IIb and IIIa.



(1) autosomal recessive condition, with consanguinity relatively common

(2) heterozygous patients do not show significant bleeding

(3) homozygous patients may have a bleeding condition from birth that can range from subclinical to severe


Common bleeding symptoms:

(1) recurrent mucocutaneous hemorrhage

(2) easy bruisability or purpura

(3) epistaxis

(4) gingival bleeding

(5) menorrhagia in women

(6) gastrointestinal bleeding (12%)


Rare findings

(1) hemarthroses (3%)

(2) deep visceral hematomas (1%)

(3) intracranial hemorrhage (2%)

(4) hematuria (6%)


Laboratory findings:

(1) normal platelet count

(2) normal platelet morphology

(3) prolonged bleeding time

(4) absent or diminished clot retraction

(5) absent or severely diminished platelet aggregation to ADP, collagen, thrombin and epinephrine

(6) platelet aggregate with ristocetin or von Willebrand's factor, but this may be reversible


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