Hemolytic disorders/ thromboembolic disease Flashcards
What tends to be the dominant influence on thrombosis specifically in the heart and arterial circulation?
Endothelial injury causing loss of the pathways which normally stop hemorrhaing from wounds.
What is the Factor V Leiden mutation?
A point mutation substituting arginine at position 506 of Factor V protein causing resistance to degradation by activated protein C.
What are some of the symptoms commonly seen in those with Factor V Leiden mutations?
Recurrent DVT (Deep vein thrombosis) Miscarriages
What is seen in antiphospholipid antibody syndrome?
Whats observed in vitro vs in vivo?
Serum antibodies destroy anionic phospholipids (commonly seen on platelets!)
In vitro it causes an inhibition of clotting due to interference of phospholipid complex assembly.
In vivo it causes a hypercoaguable state.
What disease can antiphospholipid antibody syndrome be seen in?
Can occur with or without pre existing pathologies but is most commonly seen with systemic lupus.
What are some clinical manifestations of Antiphospholipid antibody syndrome?
Recurrent venous or arterial thrombi
Repeated miscarriages
Cardiac Valve Vegetations
Thrombocytopenia (Low platelet count)
What does turbulence cause in the vasculature?
Pockets of stasis that:
Prevent dilution of clotting factors
Prevent inflow clotting inhibitors
Promote endothelial cell activation.
Where do arterial thrombi and venous thrombi typically occur
Arterial thrombi are usually at sites of endothelial injury
Venous thrombi are usually at sites of stasis
Its important to recall that thrombi firmly attach to the vessel, and when erupted cause instant thrombosis.
When ranking arterial thrombi areas of incidence where are they most likely to occur/
Most likely to occur in the coronary arteries, then the cerebral arteries followed last by the femoral arteries.
You see a thrombi appearing grey-white and friable, what sort is this likely?
Likely an arterial thrombi composed of platelets, fibrin, erythrocyte, or leukocytes.
When is stinting recommended for abdominal aorta aneurysms and what is the associated mortality rate?
Stinting is recommended for the abdominal aorta when it exceeds greater than 5cm in diameter.
The associated mortality rate is 75%.
Alternating layering of platelets, fibrin, and erythrocytes should make you think of what?
“Lines of Zahn in a Thrombus”
Arterial thrombosis in which the layering is a direct result of pressure in the arteries.
Why are phlebothrombosis asymptomatic in 50% of patients?
Phlebothrombosis are typically occurent in the Deep veins of the lower extremity. The collateral circulation is able to help mask the symptoms.
What causes Trousseau syndrome/migratory thrombophlebitis?
A serine protease released by malignant tumor cells that activate factor 10.
Tumor cells also release plasma membrane vesicles exhibiting procoagulant activity.
Think cancer!!!!!*
What is Trousseaus syndrome?
Thrombosis systemically that appears then leaves reappearing at another body site.
Where do venous thrombi typically travel to?
Travel to the right side of the heart where they will likely embolize in the lungs.
Virtually all emboli originate from…
Thrombus!
Unless otherwise states all emboli are assumed to be thromboembolus.
Whats the difference between an embolus and an embolism
An embolus is a clot traveling within the body.
And embolism is a clot that blocks the blood supply.
What structure serves as the number 1 source of systemic thromboembolisms?
Intracardial mural thrombi.
What is a Mural Thrombi?
An arterial thrombosis in cardiac chambers due to myocardial infarction, ulcerated artherosclerotic plaque or aneurysmal dilation.
What is a paradoxical embolism?
A venous embolism gets into the systemic blood instead of the lungs due to an inter-atrial or inter-ventricular defect.
What is the term used to describe a pulmonary embolus that lodges across the vessel bifurcation?
This is a saddle embolus which often leads to instant death.
This is classified as a large emboli (5%)
Small to medium sized emboli are likely to cause what sort of disfunction?
More likely to cause nfarction seen in patients with heart or lung disease
How do Saddle emboli kill pt?
Cause right sided heart failure due to blockage within the lung.
In a pulmonary emboli one would expect the V/Q to be thrown off in what way?
The perfusion (Q) would be greatly deminished by the clot.
When are fat emboli most commonly seen?
Fractures of the long bones
Soft tissue trauma (Crush trauma)
Burns
Usually have no effect but in select few will cause fat embolism syndrome.
What is the Oil red O stain used for?
Looks to reveal fat embolism!
Diffuse petechial rash, pulmonary insuficiency, thrombocytopenia and anemia are all symptoms of what sort of emboli?
All symptoms of a Fat embolism.
How many CCC’s of air are typically required to generate an air embolism?
Typically requires around 100 CC’s
