Circulatory Pathology

Question 1

Absence or decreased expression of the GPIb, autosomal recessive, mechanism of disease and name of disease

Answer 1

• Bernard-soulier syndrome

- defective platelet adhesion

Question 2

Deficiency of GPIIb-IIIa, autosomal recessive. Mechanism of disease and name of disease.

Answer 2

• glanzmann thrombasthenia

- defective platelet aggregation

Question 3

Target of aspirin and what factor that participate in platelet aggregation is affected

Answer 3

• irreversibly acetylates cyclooxygenase

- ⬇️ platelet production of thromboxane A2

Question 4

Thrombotic thrombocytopenia purpura (TTP), mechanism of disease

Answer 4

Deficiency or inhibition of ADAMTS13 ▶️ cleave large multimers of Von Willebrand factor

Question 5

Microorganism and toxin associated with hemolytic uremic syndrome

Answer 5

E. coli 0157:H7 Verotoxin producing

Question 6

What do you use to monitor the warfarin and heparin therapy?

Answer 6

• warfarin (extrinsic) - prothrombin time

- heparin (intrinsic) - partial thromboplastine time

Question 7

Treatment of Von Willebrand disease

Answer 7

mild cases (type 1): desmopreessin ▶️ release vWF from Weibel-Palade bodies of endothelial cells

Question 8

Abnormal platelet response to ristocetin, normal platelet count, prolonged bleeding time, normal PT, often prolonged PTT

Answer 8

Von Willebrand disease

Question 9

Why is there an elevation of fibrin split product (D-dimers) in DIC?

Answer 9

Microthormbi ▶️ activation of plasmin fibrinolysis ▶️ fibrin split products ▶️ (-) thrombi, platelet aggregation, fibrin polymerization ▶️ bleeding

Question 10

Normal platelet count, prolonged bleeding time, normal PT and often prolonged PTT, abnormal platelet response to ristocetin

Answer 10

Von Willebrand disease

Question 11

Meningococcal septic shock which causes bilateral hemorrhagic infarction and acute adrenal insufficiency

Answer 11

Waterhouse-Friderichsen syndrome

Question 12

In gray platelet syndrome, patients lack alpha granules. The cross-linking of platelets through the Gp IIb-IIIa receptor would be reduced because of lack of which factor?

Answer 12

von Willebrand factor

*normally: cross-linking and stabilizing of the platelet plug and stabilizing circulating factor VIII. Template bleeding time, which measures formation of the primary platelet plug, will be prolonged

Question 13

Consequence of factor V resistant (change Gln by arg) to cleaved by protein C

Answer 13

Recurrent deep vein thrombosis (factor V Leiden)

*approximately 60% of the patients with recurrent deep vein thromboses are carriers of factor V Leiden.