Hereditary and Acquired Thrombotic Disorders Flashcards
What are the three components of Virhow’s triad?
- Decreased blood flow
- Inflammation of or near the blood vessels
- Intrinsic alterations in the nature of the blood itself
What are arterial thrombi composed of?
Primarily of aggregated platelets, containing small amounts of fibrin and few red cells making them appear a white color.
When do venous thrombi form?
They typically develop under conditions of slow blood flow.
What are venous thrombi composed of?
Large amounts of fibrin containing numerous red cells (red thrombi)
What is phlegmasia cerulean dolens?
An extremely swollen, blue, and painful leg due to the complete obstruction of a proximal vein
Lesser degrees of obstruction of a leg can produce what symptoms?
Pain, pitting edema of the distal extremity, and a warm, dusky, reddish-blue discoloration of the skin
Describe postthrombotic syndrome.
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.
What are the classical signs and symptoms of a PE?
Sudden chest pain, dyspnea, anxiety, cough, syncope and cyanosis.
Uncommonly hemptysis
Patients can present with cardiac arrest and death
What are the sighs and symptoms someone can develop with recurrent PE?
Chronic dyspnea
Chronic pulmonary hypertension
Elevated right heart pressure
How do you diagnose a PE?
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)
What would you use to treat an arterial thrombi in an acute setting?
Heparin and a fibrinolytic agent such as tPA
Long term: antiplatelet since arterial thrombi are mostly platelets
- –Aspirin
- –thienopyridines
- –clopidogrel
- –glycoprotein IIb/IIIa inhibitors
What would you use to treat a venous thrombi in an acute setting?
Unfractionated r low molecular weight heparin
Long-term: low molecular weight heparin or warfarin
What is the inheritance pattern of Facto V Leiden?
Autosomal dominant
What phenotype is produced in Factor V Leiden?
Partial resistance to inactivation through proteolytic cleavage by protein C leading to increased risk of thrombosis
Should asymptomatic Factor V Leiden patients be treated?
No they should be informed of what activities cause increase risk
What is the inheritance pattern of prothrombin G20210A?
Second most common inherited predisposition to hypercoagulability and has an autosomal dominant pattern
What is elevated in patients with prothrombin G20210A?
Elevated concentrations of plasma prothrombin
What is the role of protein C?
When it becomes activated it inactivates factor Va and VIIIa to inhibit coagulation
What is the inheritance pattern of Protein C Deficiency?
Autosomal dominant
Heterozygotes make ____% of normal protein C levels?
50%
What happens in homozygous deficient protein C patients?
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
A patient with protein C deficiency is at risk of developing what condition when taking warfarin?
Skin necrosis
What is the inheritance pattern of protein S?
Autosomal dominant. They have risk of venous and arterial thrombosis
What are some similarities between protein C deficiency and protein S deficiency?
Both can be seen with neonatal purpura fulminans and warfarin-induced skin necrosis
same treatment
What is the role of antithrombin III?
Regulates coagulation by inactivating thrombin as well as factors Xa, IXa, XIa and XIIa
What is the inheritance pattern of antithrombin deficiency?
Autosomal dominant
What are the symptoms of someone who is homozygous deficient for antithrombin?
Fatal in utero
Can you use heparin to treat antithromin deficiency?
No because lack of AT-III limits the therapeutic effectiveness of heparin
Should asymptomatic antithrombin deficient patients be put on anticoagulant therapy?
no
Describe hyperhomocyteinemia
- Inherited or acquired
- incereased risk for thrombosis may be enhanced platelet activation and adhesiveness due to endothelial cell injury
Describe Increased factor VIII activity.
Increased risk of VTE
Describe impaired fibrinolysis.
Can lead to increased risk of thrombosis
Does Lupus antivoagulants, anticardiolipin antibodies, and beta-2 glycoprotein 1 antibodies lead to venous or arterial thrombosis?
Both
Do oral contraceptives increase your risk of developing a clot?
Yea
