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2nd U2 Path > Secondary Hemostasis and Related Disorders > Flashcards

Secondary Hemostasis and Related Disorders Flashcards

1
Q

What is the purpose of secondary hemostasis?

A

Stabilizes the weak platelet plug via the coagulation cascade

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2
Q

What is generated during the coagulation cascade?

A

Thrombin

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3
Q

What does thrombin convert during the coagulation cascade?

A

Fibrinogen→ fibrin in the platelet plug

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4
Q

What is the outcome of secondary hemostasis?

A

Platelet-fibrin thrombus stabilizing the platelet plug

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5
Q

Factors of the coagulation pathway are produced by the liver in an inactive form. What are the 3 requirements for activation of these factors?

A

1) Exposure to an activating substance
2) Phospholipid surface of platelets
3) Calcium (derived from platelet dense granules)

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6
Q

What are the common clinical features seen with disorders of secondary hemostasis?

A

Deep tissue bleeding into muscles and joints (hemarthrosis) and rebleeding after surgical procedures

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7
Q

What does prothrombin time (PT) measure?

A

Measures extrinsic (Factor VII) and common (factors II, V, X and fibrinogen) pathways of the coagulation cascade

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8
Q

What does partial throboplastin time (PTT) measure?

A

Intrinsic (factors XII, XI, IX, VIII) and common (factors II, V, X, and fibrinogen) pathways of the coagulation cascade

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9
Q

Factor VIII deficiency that can either be X-linked recessive or arise from a new mutation without family history…

A

Hemophilia A

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10
Q

What is the common presentation for Hemophilia A?

A

Deep tissue, joint, and postsurgical bleeding

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11
Q

What are the findings on laboratory studies of a patient with Hemophilia A with respect to PT, PTT, FVIII, platelet count, and bleeding time?

A 
PT: normal
PTT: elevated
FVIII: decreased
Platelet count: normal
Bleeding time: normal
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12
Q

What is the treatment for Hemophilia A?

A

Give recombinant FVIII

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13
Q

Factor IX deficiency that resembles Hemophilia A, except Factor IX is deficient…

A

Hemophilia B

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14
Q

Disease involving an acquired antibody against a coagulation factor that results in impaired factor function…

A

Coagulation factor inhibitor

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15
Q

What is the most common antibody involved in coagulation factor inhibitors?

A

Anti-FVIII

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16
Q

How is a coagulation factor inhibitor anti-FVIII differentiated from Hemophilia A?

A

A mixing study

Mix normal plasma with patient’s plasma—If PTT is corrected then Hemophilia A and if PTT remains elevated then its most likely a coagulation factor inhibitor against FVIII

17
Q

The most common inherited coagulation disorder…

A

Von Willebrand disease

18
Q

How does Von Willebrand disease present?

A

With mild mucosal and skin bleeding

19
Q

What causes the mild mucosal and skin bleeding seen with Von Willebrand disease?

A

Low vWF impairing platelet adhesion

20
Q

What is found on the labs of a patient with Von Willebrand disease with respect to bleeding time, PTT, and PT?

A

Bleeding time: increased
PTT: increased—vWF is needed to stabilize FVIII
PT: normal

21
Q

What abnormal test is found when performed on patients with Von Willebrand disease?

A

Ristocetin test

Ristocetin induces platelet agglutination by causing vWF to bind platelet GPIb—No vWF=impaired agglutination→ abnormal test

22
Q

What is the treatment for Von Willebrand disease?

A

Desmopression and ADH analog

23
Q

Desmopression is used for the treatment of Von Willebrand disease. What is the mechanism of action of desmopression?

A

Increases vWF released from Weibel-Palade bodies of endothelial cells

24
Q

What is disrupted when an individual has a vitamin K deficiency?

A

Disruption of multiple coagulation factors

25
Q

Where and how is Vitamin K activated?

A

Activated in the liver by epoxide reductase

26
Q

What is the function of activated vitamin K?

A

Gamma carboxylates factors II, VII, IX, X, proteins C and S

This is necessary for factor function

27
Q

What are the 3 most common causes of Vitamin K deficiency?

A
  • Newborn
  • Long-term antibiotic therapy
  • Malabsorption
28
Q

Why is vitamin K deficiency common in newborns?

A

Lack of GI colonization by bacteria that normally synthesize vitamin K

29
Q

What is given to newborns to prevent hemorrhagic disease?

A

Vitamin K injection

30
Q

How does long-term antibiotic therapy cause a vitamin K deficiency?

A

The antibiotic disrupts the vitamin K-producing bacteria in the GI tract

31
Q

How does malabsorption cause a vitamin K deficiency?

A

Malabsorption causes a deficiency of fat-soluble vitamins—AEDK

32
Q

How does liver disease cause abnormal secondary hemostasis?

A

Decreased production of coagulation factors

Decreased activation of vitamin K by epoxide reductase

33
Q

Which laboratory test is used to follow the effect of liver failure on secondary hemostasis?

A

PT

34
Q

How does a large-volume transfusion cause abnormal secondary hemostasis?

A

Dilutes the coagulation factors

2nd U2 Path (31 decks)

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