Glanzmann's thrombasthenia is an abnormality of the platelets. It is an extremely rare autosomal recessive coagulopathy in which the platelets lack glycoprotein IIb/IIIa.
Hence, no fibrinogen bridging can occur, and bleeding time is significantly prolonged.
There is a normal platelet count and morphology, and normal (but possibly reversible) platelet agglutination by ristocetin and VWF. However there is absent or severely diminished platelet aggregation in response to natural agonists - ADP, collagen, thrombin, & epinephrine, and absent or diminished clot retraction in most patients
However these days flow cytometry using monoclonal antibodies to GPIIb- IIIa is the current method of choice for diagnostic confirmation. Times have changed…..
No comments:
Post a Comment