Coagulopathies 9-16-14 Flashcards
What are the 3 phases in hemostasis?
Vascular (vasoconstriction), platelet (plug), and plasma (coagulation)
What is the average life span of platelets?
5-9 days
*Define platelets (look up definition)
Small, disc shaped cell fragments that assist in blood clotting
*Define von Willebrand factor (vWE)
A plasma protein that is important in the platelet phase, especially adhesion and aggregation
(congenital is caused by mutations in the vWF gene)
*A patient presents with mucocutaneous bleeding in gums. What disorder is responsible?
Primary hemostasis (platelets and VW)
*A patient presents with abnormal bleeding into their joint spaces. What disorder is responsible?
Defects in coagulation factors
?Describe the steps involved in bleeding and clotting (recheck)
- Platelet phase
- Platelets
- Platelet adhesion
- Platelet activation
- Platelet aggregation
- Primary hemostatic platelet plug
a) What is the most common inherited bleeding disorder?
b) Who is affected
a) von Willebrand disease
b) Males and Females equally
It a patient has a defect in vWF, what also might be affected?
Factor 8
What type of vWF is autosomal dominant?
Type 1
What type of vWF is a result of complete absence of vW?
Type 3
What type of vWF is a result of qualitative defect of vWF?
Type 2
*What 3 tests are used to screen for vWD?
- Plasma von Willebrand factor antigen
- Plasma VWF activity
- Factor VIII activity
What is the treatment for vWF type 1 and some type 2?
- Stimate nasal spray
- Synthetic hormone
What is the treatment for vWF type 3 or type 1 trauma patients?
Humate P
Describe hemophilia
Sex linked inherited deficiency of Factor VIII (A) or Factor IX(B)
What are the laboratory findings that are used to diagnose a patient with hemophilia A, B?
NL platelet count
Nl PT
Prolonged aPTT
What lab test is used to diagnose Hemophilia?
Specific factor assays (VIII, IX)
What treatment is used for hemophilia?
- Factor replacement, pain control, rest or immobilize joint
- Prophylaxis/on demand
- Home therapy
*Formulate a differential diagnosis for a bleeding disorder (check with class notes)
- primary hemostasis (PLT number or function or vW disease)
- secondary hemostasis (Hemophilia, liver, drugs, vitamin K )
What are the 3 main causes of thrombosis (Virchow’s triad)?
- Reduced blood flow (stasis)
- Blood hypercoagulability
- Vascular wall abnormalities
- Define thrombolysis
Lysis of blood clots by pharmacological means
*Define thrombosis
Local coagulation or clotting of the blood in the veins or arteries
What is the difference between arterial and venous thrombosis?
- Venous thrombosis- coagulation system abnormalities (DVT)
- Arterial thrombosis- platelets play the predominant role (HA)
*Describe Factor V Leiden (FVL) disorder
-Activated protein C inhibitor of the coagulation system that that reduces the amount of factor 5 and 8
What is the most common inherited throbophilia?
Factor V Leiden(FVL) disorder
How do you diagnose for FVL?
Genetic testing
How long must a patient be off of coumadin to test for C & S deficiency?
2-3 weeks before testing
What is nephrotic syndrome?
Loss of natural anticoagulants in urine
*Discuss the role of malignancy in hypercoagulation
- 20% of all VTEs occur in cancer
- 10% of all unprovoked VTEs will be diagnosed with cancer the year after diagnosis
*Discuss the role of contraceptive pills (OCP) in hypercoagulation
- Estrogen OCPs increase risk 3-5-fold
- Absolute risk is 1 in 3000/year
- Risk is further incr. by a # of risk factors (obesity, smoking, family history of VTE, presence of thrombophilias)
- Obesity multiplies risk to 20-30 fold, or 1 in 500/yr
*Discuss the role of Nephrotic syndrome in hypercoagulation
- Loss of natural anticoagulants
- Multiple other abnormalities (increased PLT aggregation, impaired fibrinolysis etc)
- Increased risk of thrombosis
*Discuss the role of Pregnancy in hypercoagulation
- 4-fold increase during pregnancy & post-partum
- 5-fold increase postpartum vs pregnancy
- Stasis, hypercoagulability (VII,VIII,X,Fibrinogen, vWF increase, decrease in protein S)
- Usually prophylaxis in high risk thrombophilia
