Patients with type 3 VWD may develop antibodies to VWF after replacement therapy, which complicates management. The most common hereditary intrinsic platelet disorders are a group of mild bleeding disorders that may be considered disorders of amplification of platelet activation. Because aspirin's effect can persist for several days, it must be confirmed that a patient has not taken aspirin for several days before testing to avoid confusion with a hereditary platelet defect. Thrombasthenia is a rare hereditary platelet defect that affects platelet surface membrane glycoproteins. It is an autosomal recessive disorder.
Consanguinity is common in affected families.
Their platelets, lacking the membrane glycoprotein GP IIb-IIIa, fail to bind fibrinogen during platelet activation and thus fail to aggregate. Bernard-Soulier syndrome is another rare autosomal recessive disorder that affects surface membrane glycoproteins. Unusually large platelets are present that do not agglutinate with ristocetin but aggregate normally with the physiologic aggregating agents ADP, collagen, and epinephrine. Therefore, the platelets do not adhere normally to subendothelium despite normal VWF levels in plasma. Serious bleeding in a patient with an intrinsic platelet disorder may require platelet transfusion.