Hemostasis and Coagulation Factors: The Cascade System Flashcards

1
Q

What are clotting factors that circulate in the blood as distinct entities in an inactive form?

A

Zymogens

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2
Q

When this factor becomes activated, it’s referred to as what?

A

Serine protease

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3
Q

What binds to a particular serine protease to stabilize and increase reactivity?

A

Cofactors

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4
Q

This factor is a substrate for thrombin and polymerizes to form fibrin

A

Factor I/Fibrinogen

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5
Q

This factor is a cofactor for II → IIa and is a part of the prothrombinase complex

A

Factor V/Labile factor

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6
Q

This factor is a cofactor for X → Xa

A

VIII:C

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7
Q

This factor is a carrier protein for VIII:C and is needed for platelet adhesion to collagen

A

vWF

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8
Q

This factor is a transglutaminase which converts loose hydrogen bonds of fibrin monomers to covalent bonds

A

XIII/Fibrin stabilizing factor

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9
Q

This factor is a serine protease and a precursor to thrombin

A

Factor II/Prothrombin

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10
Q

This factor is a serine protease and activates X and IX (in vivo)

A

Factor VII/Stabilizing factor

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11
Q

This factor is a serine protease and w/ help of the cofactor VIIIa, it converts X → Xa

A

Factor IX/Christmas factor

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12
Q

This factor is a serine protease that is a part of the prothrombinase complex which converts II → IIa

A

Factor X/Stuart-Prower factor

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13
Q

This factor is a serine protease that converts IX → IXa

A

Factor XI/Plasma thromboplastin antecendant

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14
Q

This factor is a serine protease (in vivo) that is activated in the presence of HMWK and PK to activate XI → XIa

A

Factor XII/Hageman factor

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15
Q

This factor is a serine protease (in vivo) and w/ the help of cofactor HMWK it activates XII→ XIIa

A

Factor PK/Fletcher factor

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16
Q

This factor is a cofactor that participates in the (in vitro) contact activation of XI → XIa and activates the kinin system

A

Factor HMWK/Fitzgerald factor

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17
Q

Which 12 factors are synthesized in the liver?

A

Factors I, V, VIII:C, XIII, II, VII, IX, X, XI, XII, PK, and HMWK

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18
Q

Which cofactors are synthesized in the endothelial cells and megakaryocytes?

A

vWF

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19
Q

Which enzyme is generated from the coagulation pathway that is needed to convert fibrinogen to fibrin?

A

Thrombin

20
Q

Which site provides the surface for the coagulation cascade?

A

PF3 (PL) (assembly molecule)

21
Q

Initiation of coagulation by ____ is sufficient to initiate activation of both pathways

A

TF or Factor VII complex

22
Q

Which pathway is being tested by prothrombin time (PT)?

A

Extinsic pathway

23
Q

Which pathway is being tested by Activated Partial Thromboplastin Time (APTT)?

A

Intrinsic pathway

24
Q

List the correct order of activation of factors for in vitro testing by the Prothrombin Time (PT)

A
  • VII
  • X
  • V
  • II
  • I
25
Q

List the correct order of activation of factors for in vitro testing by the Activated Partial Thromboplastin Time (APTT)

A
  • XII (plus Fletcher and Fitzgerald )
  • XI
  • IX
  • VIII
  • X
  • V
  • II
  • I
26
Q

List the correct order of activation factors for the common pathway

A

X, V, II, I

27
Q

What is the role of the prothrombinase complex in the common pathway?

A

Macromolecular complex which forms on the surface of the platelets; great amplifier of thrombin activation
- (made up of Xa, Va, PL, and calcium)

28
Q

How is the unstable, soluble fibrin monomer formed in the common pathway?

A
  • Fibrinogen is composed of three pairs of polypeptide chains (α, ß, and γ)
  • Thrombin cleaves a portion of the α and ß to form fibrinopeptides A and B
  • After the cleavage, the fibrinogen molecule is called a fibrin monomer
  • Fibrin monomer quickly polymerizes (line up side by side) to form fibrin (unstable w/ loose hydrogen bonds)
29
Q

What factor is responsible for forming the stable, insoluble fibrin clot?

A

Factor XIII and Ca2+ convert loose hydrogen bonds to stronger covalent (cross-linked) bonds

30
Q

Four hemostatic actions of thrombin

A
  • Converts fibrinogen to fibrin
  • Activates factors XI, V, VIII, and XIII
  • Induces platelet aggregation
  • Binds the endothelial cell membrane protein, thrombomodulin, to activate the protein C pathway
31
Q

Nomenclature of the two portions of the Factor VIII complex

A

vWF and VIII:C

32
Q

Site of synthesis of the two portions of the Factor VIII complex

A
  • vWF: endothelial cells and megakaryocytes

- VIII: liver

33
Q

Principle biologic activity of the two portions of the Factor VIII complex

A
  • vWF: carrier for Factor VIII

- VIII: cofactor for X → Xa

34
Q

Clinical disorder due to deficiency of hte two portions of the Factor VIII complex

A
  • vWF: von Willebrand’s Disease

- VIII: Hemophilia A

35
Q

Which portion of the Factor VIII complex is responsible for normal bleeding time?

A

vWF

36
Q

Which portion of the Factor VIII complex is responsible for normal platelet adhesion?

A

vWF

37
Q

Which portion of the Factor VIII complex is responsible for ristocetin cofactor activity?

A

vWF

38
Q

Which coagulation factors are present in the fibrinogen group?

A
  • I
  • V
  • VIII
  • XIII
39
Q

Which coagulation factors are present in the prothrombin group?

A
  • II
  • VII
  • IX
  • X
40
Q

Which coagulation factors are present in the contact group?

A
  • XI
  • XII
  • PK
  • HMWK
41
Q

Which coagulation factors are consumed or destroyed during clotting?

A

I, V, VIII (VII:C/vWF), XIII, II

fibrinogen group AND II

42
Q

Which coagulation factors are dependent upon vitamin K for synthesis?

A

II, VII, IX, X (prothrombin group)

43
Q

Which coagulation factors are destroyed by plasmin?

A

I, V, VIII, XIII (fibrinogen group)

44
Q

Which coagulation factors are heat labile?

A

I, V, VIII, XIII (fibrinogen group)

45
Q

What is another name for coagulation/clotting factors?

A

Procoagulant

46
Q

What are plasma GPs that serve as inhibtors to coagulation?

A

Anticoagulants

47
Q

Describe what happens in contact activation?

A

Contact w/ a negatively charged surface is one means to activate the intrinsic coagulation pathway

  • Examples: prosthetic valves, stents (in vivo); nonsiliconized glass, microsilica, elagic acid (in vitro)
  • Contact factors, EXCEPT IX (!), do not normally have an in vivo procoagulant function