Disorders of Platelet Aggregation (GP IIb/IIIa Function)

Question 1

biochemical lesion responsible for the disorder is a deficiency or abnormality of the platelet membrane glycoprotein (GP) IIb/IIIa

Answer 1

Glanzmann Thrombasthenia

Question 2

t/f.
Glanzmann Thrombasthenia

Individuals with type 2 disease have more GP IIb/IIIa complexes (10% to 20% of normal) than those with type 1 disease (0% to 5% of normal), although there is considerable variability within each subdivision

Answer 2

true

Question 3

Glanzmann Thrombastenia

patients with type _ disease are less affected by abnormal clot retraction and fibrinogen binding.

Answer 3

2

Question 4

code for GP IIb/IIIa

Answer 4

ITGA2B and ITGB3 genes present on chromosome 17

Question 5

Rarely, _ and _ may be seen with some of the mutations in these genes in GT

Answer 5

thrombocytopenia and large platelets

Question 6

The typical laboratory features of GT include a _, _ , and a lack of platelet aggregation in response to all platelet activating agents

Answer 6

normal platelet count, normal platelet morphology

Question 7

Treatment of bleeding episodes in patients with Glanzmann thrombasthenia requires the

Answer 7

transfusion of normal platelets

Question 8

Thus in GT patients who are bleeding, to minimize or prevent human leukocyte antigen (HLA) alloimmunization, it is recommended to use _ or _

Answer 8

pre-storage, leukocyte-reduced apheresis platelets or HLA-matched donor platelets.

Question 9

Glanzmann Thrombocytopenia

This rare disorder manifests itself clinically in the neonatal period or infancy, occasionally with bleeding after circumcision and frequently with _ and _. Hemorrhagic manifestations include _

Answer 9

epistaxis and gingival bleeding;

petechiae, purpura, menorrhagia, gastrointestinal bleeding, and hematuria