Platelet disorders are another important cause of bleeding. Inherited defects of platelet receptors

include defects of GpIIb/IIIa (Glanzmann’s thrombasthenia), characterized by impaired platelet binding

to vWF, fibrinogen, and fibronectin. In patients with defects in GpIb (Bernard–Soulier syndrome), the

absolute number of platelets is decreased, the platelets are larger, and platelet aggregation and adhesion

are abnormal. Acquired deficits occur in uremia, both GpIb and GpIIb/IIIa receptors are defective,

resulting in impaired adhesion and aggregation. Acquired deficits also occur in patients who previously

received platelet transfusions and then acquire immune-mediated antiplatelet antibodies.

Abnormalities in Fibrinolysis

Abnormalities in fibrinolysis may play a role in abnormal bleeding disorders. Genetic or acquired

deficiencies in α2

-AP may be associated with bleeding, whereas deficiencies in factor XIII (fibrin

stabilizing factor) may lead to highly lysable clot. α2

-AP deficiency is treated with ε-aminocaproic acid

or tranexamic acid. Homozygous patients with factor XIII deficiency and less than 1% of normal plasma

activity often show bleeding from the umbilical cord at birth, bleeding after trauma or surgery, and

delayed bleeding 24 to 36 hours later. Screening test results include a shortened euglobulin lysis time. A

specific assay for factor XIII activity exists. Treatment consists of FFP, cryoprecipitate, and factor XIII

concentrates.

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