Hemolytic disorders/ thromboembolic disease

Question 1

What tends to be the dominant influence on thrombosis specifically in the heart and arterial circulation?

Answer 1

Endothelial injury causing loss of the pathways which normally stop hemorrhaing from wounds.

Question 2

What is the Factor V Leiden mutation?

Answer 2

A point mutation substituting arginine at position 506 of Factor V protein causing resistance to degradation by activated protein C.

Question 3

What are some of the symptoms commonly seen in those with Factor V Leiden mutations?

Answer 3

Recurrent DVT (Deep vein thrombosis)
Miscarriages

Question 4

What is seen in antiphospholipid antibody syndrome?

Whats observed in vitro vs in vivo?

Answer 4

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.

Question 5

What disease can antiphospholipid antibody syndrome be seen in?

Answer 5

Can occur with or without pre existing pathologies but is most commonly seen with systemic lupus.

Question 6

What are some clinical manifestations of Antiphospholipid antibody syndrome?

Answer 6

Recurrent venous or arterial thrombi
Repeated miscarriages
Cardiac Valve Vegetations
Thrombocytopenia (Low platelet count)

Question 7

What does turbulence cause in the vasculature?

Answer 7

Pockets of stasis that:
Prevent dilution of clotting factors
Prevent inflow clotting inhibitors
Promote endothelial cell activation.

Question 8

Where do arterial thrombi and venous thrombi typically occur

Answer 8

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.

Question 9

When ranking arterial thrombi areas of incidence where are they most likely to occur/

Answer 9

Most likely to occur in the coronary arteries, then the cerebral arteries followed last by the femoral arteries.

Question 10

You see a thrombi appearing grey-white and friable, what sort is this likely?

Answer 10

Likely an arterial thrombi composed of platelets, fibrin, erythrocyte, or leukocytes.

Question 11

When is stinting recommended for abdominal aorta aneurysms and what is the associated mortality rate?

Answer 11

Stinting is recommended for the abdominal aorta when it exceeds greater than 5cm in diameter.

The associated mortality rate is 75%.

Question 12

Alternating layering of platelets, fibrin, and erythrocytes should make you think of what?

Answer 12

“Lines of Zahn in a Thrombus”

Arterial thrombosis in which the layering is a direct result of pressure in the arteries.

Question 13

Why are phlebothrombosis asymptomatic in 50% of patients?

Answer 13

Phlebothrombosis are typically occurent in the Deep veins of the lower extremity. The collateral circulation is able to help mask the symptoms.

Question 14

What causes Trousseau syndrome/migratory thrombophlebitis?

Answer 14

A serine protease released by malignant tumor cells that activate factor 10.

Tumor cells also release plasma membrane vesicles exhibiting procoagulant activity.

Think cancer!!!!!*

Question 15

What is Trousseaus syndrome?

Answer 15

Thrombosis systemically that appears then leaves reappearing at another body site.

Question 16

Where do venous thrombi typically travel to?

Answer 16

Travel to the right side of the heart where they will likely embolize in the lungs.

Question 17

Virtually all emboli originate from…

Answer 17

Thrombus!

Unless otherwise states all emboli are assumed to be thromboembolus.

Question 18

Whats the difference between an embolus and an embolism

Answer 18

An embolus is a clot traveling within the body.

And embolism is a clot that blocks the blood supply.

Question 19

What structure serves as the number 1 source of systemic thromboembolisms?

Answer 19

Intracardial mural thrombi.

Question 20

What is a Mural Thrombi?

Answer 20

An arterial thrombosis in cardiac chambers due to myocardial infarction, ulcerated artherosclerotic plaque or aneurysmal dilation.

Question 21

What is a paradoxical embolism?

Answer 21

A venous embolism gets into the systemic blood instead of the lungs due to an inter-atrial or inter-ventricular defect.

Question 22

What is the term used to describe a pulmonary embolus that lodges across the vessel bifurcation?

Answer 22

This is a saddle embolus which often leads to instant death.

This is classified as a large emboli (5%)

Question 23

Small to medium sized emboli are likely to cause what sort of disfunction?

Answer 23

More likely to cause nfarction seen in patients with heart or lung disease

Question 24

How do Saddle emboli kill pt?

Answer 24

Cause right sided heart failure due to blockage within the lung.

Question 25

In a pulmonary emboli one would expect the V/Q to be thrown off in what way?

Answer 25

The perfusion (Q) would be greatly deminished by the clot.

Question 26

When are fat emboli most commonly seen?

Answer 26

Fractures of the long bones
Soft tissue trauma (Crush trauma)
Burns
Usually have no effect but in select few will cause fat embolism syndrome.

Question 27

What is the Oil red O stain used for?

Answer 27

Looks to reveal fat embolism!

Question 28

Diffuse petechial rash, pulmonary insuficiency, thrombocytopenia and anemia are all symptoms of what sort of emboli?

Answer 28

All symptoms of a Fat embolism.

Question 29

How many CCC’s of air are typically required to generate an air embolism?

Answer 29

Typically requires around 100 CC’s