Hereditary and Acquired Thrombotic Disorders

Question 1

What are the three components of Virhow’s triad?

Answer 1

1. Decreased blood flow
2. Inflammation of or near the blood vessels
3. Intrinsic alterations in the nature of the blood itself

Question 2

What are arterial thrombi composed of?

Answer 2

Primarily of aggregated platelets, containing small amounts of fibrin and few red cells making them appear a white color.

Question 3

When do venous thrombi form?

Answer 3

They typically develop under conditions of slow blood flow.

Question 4

What are venous thrombi composed of?

Answer 4

Large amounts of fibrin containing numerous red cells (red thrombi)

Question 5

What is phlegmasia cerulean dolens?

Answer 5

An extremely swollen, blue, and painful leg due to the complete obstruction of a proximal vein

Question 6

Lesser degrees of obstruction of a leg can produce what symptoms?

Answer 6

Pain, pitting edema of the distal extremity, and a warm, dusky, reddish-blue discoloration of the skin

Question 7

Describe postthrombotic syndrome.

Answer 7

Syndrome due to chronic venous insufficiency and chronic venostasis. Extremities become chronically swollen and painful and show dark skin discoloration. Cutaneous ulcers can also develop.

Question 8

What are the classical signs and symptoms of a PE?

Answer 8

Sudden chest pain, dyspnea, anxiety, cough, syncope and cyanosis.

Uncommonly hemptysis

Patients can present with cardiac arrest and death

Question 9

What are the sighs and symptoms someone can develop with recurrent PE?

Answer 9

Chronic dyspnea
Chronic pulmonary hypertension
Elevated right heart pressure

Question 10

How do you diagnose a PE?

Answer 10

Useful screenings are
-D-dimer-indirect measure
Very sensitive but not specific

-Venous ultrasound Doppler
>95 specificity and sensitivity

• Spiral CT
• Ventilation/perfusion (V/Q)

Question 11

What would you use to treat an arterial thrombi in an acute setting?

Answer 11

Heparin and a fibrinolytic agent such as tPA

Long term: antiplatelet since arterial thrombi are mostly platelets

• –Aspirin
• –thienopyridines
• –clopidogrel
• –glycoprotein IIb/IIIa inhibitors

Question 12

What would you use to treat a venous thrombi in an acute setting?

Answer 12

Unfractionated r low molecular weight heparin

Long-term: low molecular weight heparin or warfarin

Question 13

What is the inheritance pattern of Facto V Leiden?

Answer 13

Autosomal dominant

Question 14

What phenotype is produced in Factor V Leiden?

Answer 14

Partial resistance to inactivation through proteolytic cleavage by protein C leading to increased risk of thrombosis

Question 15

Should asymptomatic Factor V Leiden patients be treated?

Answer 15

No they should be informed of what activities cause increase risk

Question 16

What is the inheritance pattern of prothrombin G20210A?

Answer 16

Second most common inherited predisposition to hypercoagulability and has an autosomal dominant pattern

Question 17

What is elevated in patients with prothrombin G20210A?

Answer 17

Elevated concentrations of plasma prothrombin

Question 18

What is the role of protein C?

Answer 18

When it becomes activated it inactivates factor Va and VIIIa to inhibit coagulation

Question 19

What is the inheritance pattern of Protein C Deficiency?

Answer 19

Autosomal dominant

Question 20

Heterozygotes make ____% of normal protein C levels?

Answer 20

50%

Question 21

What happens in homozygous deficient protein C patients?

Answer 21

This condition leads to neonatal purpura fulminans, an often fatal disease associated with extensive venous or arterial thrombosis at birth and levels of protein C

Question 22

A patient with protein C deficiency is at risk of developing what condition when taking warfarin?

Answer 22

Skin necrosis

Question 23

What is the inheritance pattern of protein S?

Answer 23

Autosomal dominant. They have risk of venous and arterial thrombosis

Question 24

What are some similarities between protein C deficiency and protein S deficiency?

Answer 24

Both can be seen with neonatal purpura fulminans and warfarin-induced skin necrosis

same treatment

Question 25

What is the role of antithrombin III?

Answer 25

Regulates coagulation by inactivating thrombin as well as factors Xa, IXa, XIa and XIIa

Question 26

What is the inheritance pattern of antithrombin deficiency?

Answer 26

Autosomal dominant

Question 27

What are the symptoms of someone who is homozygous deficient for antithrombin?

Answer 27

Fatal in utero

Question 28

Can you use heparin to treat antithromin deficiency?

Answer 28

No because lack of AT-III limits the therapeutic effectiveness of heparin

Question 29

Should asymptomatic antithrombin deficient patients be put on anticoagulant therapy?

Answer 29

no

Question 30

Describe hyperhomocyteinemia

Answer 30

• Inherited or acquired

- incereased risk for thrombosis may be enhanced platelet activation and adhesiveness due to endothelial cell injury

Question 31

Describe Increased factor VIII activity.

Answer 31

Increased risk of VTE

Question 32

Describe impaired fibrinolysis.

Answer 32

Can lead to increased risk of thrombosis

Question 33

Does Lupus antivoagulants, anticardiolipin antibodies, and beta-2 glycoprotein 1 antibodies lead to venous or arterial thrombosis?

Answer 33

Both

Question 34

Do oral contraceptives increase your risk of developing a clot?

Answer 34

Yea