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IHO Week 6 (Final) > Thrombotic Disorders - Krafts > Flashcards

Thrombotic Disorders - Krafts Flashcards

1
Q

What are the risk factors associated with atherosclerosis?

A

Hypertension
Hyperlipidemia
Obesity
Smoking

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2
Q

What are the risk factors for Thrombosis?

A

Endothelial Damage (Atherosclerosis)
Stasis
Hypercoagulability

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3
Q

What are the risk factors associated with stasis?

A

Immobilization
Varicose Veins
Cardiac Dysfunction

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4
Q

What are the risk factors associated with Hypercoagulability?

A

Trauma/Surgery
Carcinoma
Estrogen/Postpartum
Thrombotic Disorders

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5
Q

What are the characteristics of Factor V Leiden?

A

Most common cause of unexplained thromboses

Seen in Caucasians

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6
Q

What is Factor V Leiden?

A

Point mutation in Factor V gene

Factor V can’t be turned off/can’t be cleaved by Protein C

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7
Q

How do you diagnose Factor V Leiden?

A

Genetic Testing

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8
Q

How do you treat Factor V Leiden?

A

Don’t unless there is a thrombosis.

If thrombosis, then give anti-coagulant (coumadin) for a little while or if other risk facters, anti-coagulate for life

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9
Q

What are the characteristics of Antithrombin III Deficiency?

A

Very Rare!

Lots of gene mutations exist

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10
Q

What is Antithrombin III Deficiency and what is it potentiated by?

A

ATIII is a natural anticoagulant that is potentiated by heparin

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11
Q

What is the deficiency in the ATIII gene?

A

Mutated gene produces less ATIII, but no genetic testing available

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12
Q

What is the risk of getting a clot?

A

Homozygotes: Can’t survive
Heterozygotes: Half get clots
Heparin won’t work
Antithrombin concentrates required

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13
Q

What are the characteristics of Protein C and S deficiencies?

A

Protein C deficiency is rare

Protein S deficiency is super-rare

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14
Q

What is Protein S?

A

Natural anticoagulant

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15
Q

What is Protein C?

A

Natural anticoagulant: Inactivates Va and VIIIa
Fibrinolytic: Promotes t-PA action
Anti-inflammatory: Keeps cytokines low

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16
Q

What is the outcome of Protein C and S Deficiencies?

A

Warfarin-induced skin necrosis

Purpura Fulminans

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17
Q

What’s wrong with the Protein C gene?

A

Mutated gene produces less Protein C or a defective Protein C
–> due to many different mutations

18
Q

How do you diagnose Protein C Deficiency?

A

Functional Testing

19
Q

What does coumadin inhibit?

A

Factor II, VII, IX, and X

Proteins C and S

20
Q

What is Purpura Fulminans?

A

Thrombotic State with Vascular Injury resulting in skin necrosis associated with Protein C and S deficiency and sepsis

21
Q

What is the treatment of Purpura Fulminans?

A

Protein C

22
Q

What are the characteristics of Factor II (Prothrombin) Gene Mutation?

A

Makes too much normal prothrombin

Common in Caucasians

23
Q

What are the characteristics of Hyperhomocysteinemia?

A

Too much homocysteine results in thromboses

24
Q

What does homocysteine do?

A

Converts folate to THF using B12/MS to make methionine

25
Q

What is homocysteine?

A

Amino Acid
Made from Methionine
Maintains Myelin
Converts dietary folate

26
Q

What is Homocysteinuria?

A

Rare Metabolic Disorder
Mutation in CBS (Cystathionine Beta Synthase)
Increased homocyteine in blood/urine
Increased thrombosis and premature atherosclerosis

27
Q

What is the pathogenic cause of Homocysteinemia?

A

Mutation in MTHFR gene (methylene tetrahydrofolate reductase)
B12/Folate Deficiency

28
Q

What’s so bad about homosysteine?

A

Toxic to Endothelium that forms reactive oxygen species

Interferes with nitric oxide (antithrombotic and vasodilator)

29
Q

What results from heterozygous homocysteinemia?

A

Increased thrombosis, premature atherosclerosis

30
Q

What is the acquired thrombotic disorder?

A

Antiphospholipid Antibody

31
Q

What are the characteristics of Antiphospholipid Antibodies?

A

Falsely prolonged INR

May cause thromboses

32
Q

What are Antiphospholipid Antibodies?

A

IgG antibodies against phospholipids = Inhibitors

33
Q

What are the 3 variants of Antiphospholipid Antibodies?

A

Anticardiolipin Antibodies
Lupus Anticoagulants
Antibodies against other molecules

34
Q

What coagulation tests do Antiphospholipid Antibodies screw up?

A

Bind to PTT/PT reagent
Can’t clot
Results are prolonged

35
Q

What other tests can Antiphospholipid Antibodies screw up?

A

Syphilis Test

Direct Antiglobulin Test

36
Q

In vivo, what do antiphospholipid antibodies do?

A

Promote Coagulation

37
Q

In vitro, what do antiphospholipid antibodies do?

A

Inhibit Coagulation

38
Q

Who is most likely to develop antiphospholipid antibodies?

A

Adults with autoimmune diseases

39
Q

What symptoms are associated with Antiphospholipid Antibody Syndrome?

A 
Recurrent Thrombosis
Recurrent Spontaneous Abortions
Increased risk of Stroke
Pulmonary Hypertension
Renal Failure
40
Q

How do you diagnose Antiphospholipid Antibodies?

A

PTT: Prolonged

PTT Mixing Study: not corrected

IHO Week 6 (Final) (11 decks)

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