Description

An acquired coagulopathy mimicking hereditary Glanzmann’s thrombasthenia can rarely occur.


 

Clinical features:

(1) bleeding

(2) purpura

(3) negative family history for a bleeding disorder

(4) negative personal history for a bleeding disorder

(5) negative history for antiplatelet drugs

 

The onset of the disorder may be associated with:

(1) monoclonal gammopathy

(2) lymphoproliferative disorder

(3) autoimmune disorder

(4) alloimmunization (rare)

 

Basic laboratory findings:

(1) The platelet count may be normal or decreased.

(2) The bleeding time is increased.

(3) Platelets do not aggregate if exposed to ADP, collagen or arachidonic acid.

(4) Platelets do aggregate if exposed to ristocetin (which is dependent on glycoprotein Ib).

(5) The addition of plasma from the patient can transfer the platelet defect to a normal patient.

 

If the diagnosis is suspected then testing will show antibody to GPIIb/IIIa glycoproteins on platelets.

 


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