BL Hereditary and Acquired Thrombotic disorders

Question 1

Virchows triad

Answer 1

3 factors that promote thrombosis

1. abnormal blood flow
2. endothelial injury
3. hypercoagulability

• only 1 needs to be present for thrombosis to occur

Question 2

factor V Leiden affects clotting how?

Answer 2

makes clotting harder to turn off –> hypercoagulability

Question 3

disseminated cancer affects clotting how?

Answer 3

makes you more likely to cloth

Question 4

Arterial thrombi:

• Are blood clotting factors or platelets more imp?
• Occurs under high shear stress or slow blood flow?
• Can cause ischemia or not?
• Affected by oral contraceptives?

Answer 4

Arterial:
○ Platelets more imp.
○ Occur in high shear stress
○ If large enough, cause downstream ischemia
○ No - venous thrombi affected by oral contra

Question 5

Venous thrombi:

• Are blood clotting factors or platelets more imp?
• Occurs under high shear stress or slow blood flow?
• More common in young or old?
• Affected by oral contraceptives?

Answer 5

○ Blood clotting factors - Large amounts of fibrin with lots of RBCs (red thrombi)
○ Slow blood flow (stasis)
○ More common with increased age
○ Yes to oral ;)

Question 6

three major clinical symptoms that occur when a patient suffers from an acute iliofemoral thrombosis of the leg

Answer 6

Edema with pain
Dilated superficial veins
Redness and warmth in the area

Question 7

How does Edema with pain occur after an acute iliofemoral thrombosis of the leg?

Answer 7

a. Impaired venous return - “backed up” blood

b. Pain comes from increased hydrostatic pressure

Question 8

How do Dilated superficial veins occur after an acute iliofemoral thrombosis of the leg?

Answer 8

the blood finds another route

Question 9

How does Redness and warmth in the area occur after an acute iliofemoral thrombosis of the leg?

Answer 9

Formation of thrombus triggers an inflammatory response

Question 10

Is Factor V Leiden a deficiency disorder?

Answer 10

No! - it is an Autosomal dominant structural change in factor V

Normally thrombin cleavage leads to Factor V activation (Va), and then APC inactivates Va.

In Factor V Leiden, Factor V is mutated and cannot be inactivated –> hypercoagulability (make more clotting factors!)

Question 11

How do you diagnose Factor V leiden?

Answer 11

DNA analysis

Question 12

Prothrombin Gene Mutation

• leads to increased or decreased prothrombin?
• Assoc. with venous or arterial thrombosis?
• Randomly triggered?

Answer 12

• Polymorphism leads to ↑ prothrombin in circulation
○ Remember that prothrombin leads to more thrombin, which leads to clots. BOOM.
• Associated with venous thrombosis

Most people with the mutation don’t clot - need something else to trigger your clot. Not just out of the blue

Question 13

Type I vs Type II antithrombin

Answer 13

○ Type I: ↓ level of protein

○ Type II: ↓ activity (can’t bind heparin)

Question 14

Is warfarin used to prevent additional clots?

Answer 14

Yes, but NOT break down already formed cloths

Question 15

If your patient is Protein C deficient, what drug would you give them?

Answer 15

Warfarin bc pt is hypercoagulable, but bridge the therapy with heparin to prevent warfarin induced necrosis

□ Note: warfarin not effective until turnover of the factors because warfarin inhibits activity of vit K, not the factors themselves.→ that’s why we give heparin (inactivates activated clotting factors) for ~5 days

Question 16

What factor has the shortest half life? Why is this useful?

Answer 16

VII - 5 hours

good to view a consumptive process

Question 17

Natural anticoagulants

Answer 17

AT III
Protein C
Protein S

Question 18

How do you acquire Protein S deficiency?

Answer 18

○ Pregnancy
○ Active thrombosis
○ DIC
○ Nephrotic syndrome
○ Warfarin use
○ Oral contraceptives

Question 19

Is antiphospholipid antibody syndrome an acquired hypercoagulable state or hemorrhagic state?

Answer 19

Severe hypercoagulable state

Question 20

antiphospholipid antibody syndrome

Answer 20

○ This is a syndrome where thrombotic or obstetric complications are caused by antibodies
○ Can see both venous and arterial thrombosis
○ Can see in any vascular bed, at any age, in any gender

○ Related to drug exposure, infection, and illness

Question 21

Clinical criteria for antiphospholipid antibody syndrome

Answer 21

• Vascular thrombosis:
1+ clinical episodes of thrombosis

• Complications of pregnancy:
1+ unexplained death of fetus > 0 wks
1+ premature births of neonates <10 wks

Question 22

Treating venous thrombi

- Acute vs long term

Answer 22

Use anticoagulant agents

○ Acute:
§ Unfractioned or low molecular weight heparin

○ Long term:
§ Low molecular weight heparin
§ Oral anticoagulation such as warfarin

Question 23

clinical clues suggesting an inherited hypercoagulable disorder.

Answer 23

1. first thrombosis before 50
2. get recurrent thrombosis
3. family hx
4. Neonatal thrombosis
5. idiopathic thrombosis
6. thrombosis at rare sites