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31 Cards in this Set
- Front
- Back
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Which cells synthesize vWF and where is it stored?
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Synthesized by endothelial cells and megakaryocytes
Stored in endothelial cells and platelets |
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What is the inheritance pattern of von Willebrand's disease?
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Autosomal dominant
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What is the inheritance pattern of Hemophilia A and B?
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X-linked
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What is the inheritance pattern of Hemophilia C?
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Autosomal recessive
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List some common clinical signs seen in von Willebrand's disease?
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1. Nose bleeds
2. Gum bleeding 3. Easy bruising and petechiae 4. Menorrhagia |
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Which blood type has lower levels of vWF than the other types?
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Type O
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What is the effect of stress on levels of vWF?
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vWF increases in stressful situations
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List 4 tests used to diagnose von Willebrand's disease.
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1. vWF activity (Ristocetin)
2. vWF antigen 3. vWF multimers 4. Factor VIII activity |
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What are the characteristics of Type I vWD?
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1. Heterozygous
2. Quantitative defect-- only half vWF produced |
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Which type of vWD results in a qualitative defect in von Willebrand factor?
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Type 2
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What are the characteristics of Type 3 von Willebrand's disease?
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1. Homozygous
2. Quantitative defect-- no vWF produced |
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How is the milder forms of von Willebrand's disease treated?
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DDAVP
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What is the long term treatment of von Willebrand's disease?
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Concentrated vWF
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Which 2 treatments are contraindicated in vWD?
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1. Cryoprecipitate
2. Fresh frozen plasma |
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Liver disease can cause decreased synthesis of all coagulation factors except...?
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Factors VIII and vWF
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What effect will liver disease have on PT and PTT values?
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Both PT and PTT will be prolonged
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Antibodies to specific coagulation factors most commonly effect which factors?
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VIII, IX, V, and XI
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Lupus anticoagulant involves an antibody against what?
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Protein-phospholipid
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What is the biggest problem caused by Lupus Anticoagulant-- bleeding or thrombosis?
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Thrombosis
(Patients usually don't have problems bleeding, despite prolonged PTT) |
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Which lab test will be prolonged in a patient with Lupus anticoagulant-- PT or PTT?
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PTT will be prolonged
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How are coagulation inhibitors (antibodies) measured?
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Measured in Bethesda units
1 Bathesda unit = amount of inhibitor present in plasma to decrease factor level by 50% in 2 hours |
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List 3 screening tests of DIC.
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1. PT and PTT
2. Platelet count 3. Fibrinogen |
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List 2 confirmatory tests for DIC.
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1. Fibrin split products (FSP)
2. D-dimers |
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When are coagulation and fibrinolytic factors produced by the fetal liver?
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10 weeks gestation
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When are platelets produced by the fetus during pregnancy?
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11 weeks gestation
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Why is the fetus and newborn at an increased risk for thrombosis?
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Protein C and S are significantly decreased, while factors V and VIII are not decreased
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What is the cause of Hemorrhagic Disease of the Newborn?
What is the treatment? |
Lack of Vitamin K to produce normal levels of coagulation factors
*Vitamin K supplementation |
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Why is primary hemostasis at an increased potential during pregnancy?
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1. Platelet count increased
2. Platelets more activated 3. Increased vWF (3-fold) |
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When does procoagulant activity increase during pregnancy?
When does fibrinolysis increase? |
Procoagulation activity increase ONLY at deliver (does not increase during labor)
Fibrinolysis activates during labor AND at deliver |
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Which syndrome is associated with cancer and painful and occlusive migratory thrombophlebitis?
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Trousseau's Syndrome
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What is the second leading cause of hospitalization for patients receiving chemotherapy?
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VTE
(venous thromboembolism) |
