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Blood and Lymph > Coagulation/Anticoagulation and Hemorrhagic/Thrombotic Disorders > Flashcards

Coagulation/Anticoagulation and Hemorrhagic/Thrombotic Disorders Flashcards

1
Q

Ability of the body to stop bleeding from a damaged vessel and to repair the defect such that normal blood flow to the involved area is restored

A

Hemostasis

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2
Q

__ domain contains N-terminal domain of all polypeptides

A

E

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3
Q

______ can also bind IXa (less affinity) to form a TFPI-IXa-TF-VIIa complex

A

TFPI

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4
Q

______ can bind to Xa at its active site, and the TFPI-Xa complex inactivates TF-VIIa

A

TFPI

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5
Q

______ cleaves fibrinogen and fibrin to form FDP (Fibrin Degradation Products)

A

Plasmin

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6
Q

______ cleaves fibrinogen to fibrin and activates XIII to XIIIa

A

Thrombin

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7
Q

______ is a cofactor for APC cleavage of Va and VIIIa.

A

Protein S

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8
Q

______ is the only Vitamin-K dependent factor that is not a serine protease

A

Protein S

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9
Q

______ on the platelet surface interact with collagen, laminin, and fibronectin

A

Beta-1 integrins

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10
Q

______ removes basic amino acids from the C-terminal end of fibrin, reducing plasminogen binding sites on fibrin

A

Thrombin-activatable fibrinogen inhbitor (TAFI)

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11
Q

_______ consists of central globule (__ domain) and globules on each end of the molecule (__ domains)

A

Fibrin, E, D

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12
Q

_______ activates V and VIII to Va and VIIIa

A

Thrombin

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13
Q

_______ cleaves and inactivates factors Va and VIIIa, resulting in decreased thrombin production

A

APC

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14
Q

_______ covalently crosslinks adjacent D domains to stabilize/strengthen the clot

A

XIIIa

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15
Q

_______ Factor adheres to exposed, damaged, subendothelium

A

von Willebrand

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16
Q

_______ hemostasis: activation of clotting cascade and formation of fibrin clot

A

Secondary

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17
Q

_______ hemostasis: platelet adhesion, aggregation, and activation

A

Primary

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18
Q

_______ increases release of vWF from endothelium

A

Arginine vasopressin

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19
Q

_______ inhibits extrinsic part of coagulation pathway/initiation phase

A

Tissue Factor Pathway Inhibitor (TFPI)

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20
Q

_______ is resistant to proteolytic action of APC

A

Factor V Leiden

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21
Q

_______ measures the extrinsic pathway

A

PT

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22
Q

_______ measures the intrinsic pathway

A

PTT/APTT

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23
Q

________: antibodies interfere with function of phospholipid-binding proteins involved in regulation of coagulation

A

Antiphospholipid Antibody Syndrome

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24
Q

_______: can lead to heparin resistance because heparin is a cofactor for antithrombin

A

Antithrombin Deficiency

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25
Q

_______: IgG antibody against phospholipid in the platelet membrane, causing Antiphospholipid Antibody Syndrome (APS)

A

Lupus Anticoagulant

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26
Q

_______: internal membrane tunnels though which contents of granules can be released

A

Canalicular System

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27
Q

_______: leads to elevated concentration of plasma prothrombin, autosomal dominant inheritance

A

Prothrombin mutation

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28
Q

_______: leads to partial resistance to inactivation via Protein C proteolytic cleave

A

Factor V Leiden

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29
Q

_______: mutation in Factor V with an autosomal dominant inheritance pattern

A

Factor V Leiden

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30
Q

______: activated reaction of plasma with an anionic substrate

A

APTT

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31
Q

______: addition of tissue thromboplastin to initiate clotting

A

PT

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32
Q

______: key cofactor for antithrombin, accelerating its rate of protease inactivation by 102-106 fold

A

Heparin

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33
Q

______: serine protease produced in endothelial cells that is used to activate plasmin

A

Tissue Plasminogen Activator (t-PA)

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34
Q

______: serpin that binds and inactivates t-PA (also activated u-PA)

A

Plasminogen Activator Inhibitor-1 (PAI-1)

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35
Q

_____: serpin that binds and inactivates plasmin in a 1:1 ratio

A

Alpha-2 antiplasmin

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36
Q

Activators of Plasmin

A

t-PA, u-PA

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37
Q

Antithrombin targets factors: ______.

A

II, Xa, kallikrein, XIIa, XIa, IXa, VIIa-TF complex

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38
Q

APC cleaves and inactivates factors ___ and ___, resulting in decreased thrombin production

A

Va, VIIIa

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39
Q

Arterial or Venous Thrombus? Composed of aggregated platelets, containing small amounts of fibrin and blood cells

A

Arterial

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40
Q

Arterial or Venous Thrombus? Composed of large amounts of fibrin and numerous red cells

A

Venous

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41
Q

Arterial or Venous Thrombus? Occur under conditions of high shear stress

A

Arterial

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42
Q

Arterial or Venous Thrombus? Red Thrombus

A

Venous

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43
Q

Arterial or Venous Thrombus? Slow Blood flow

A

Venous

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44
Q

Arterial or Venous Thrombus? White Thrombus

A

Arterial

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45
Q

Circulating platelets adhere via _______ on the platelet surface and vWF

A

glycoprotein 1b (GP1b)

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46
Q

Cofactor Factors

A

III, V, VIII, HMWK (High Molecular Weight Kininogen)

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47
Q

Diagnostic Tool: in vitro bleeding time measurement, can determine platelet response to agonists

A

PFA-100

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48
Q

Diagnostic Tool: measures activity of fibrinogen and sensitive to heparin or fibrin split products

A

Thrombin Time

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49
Q

Diagnostic Tool: measures entire pathway, but more sensitive to intrinsic pathway: especially XI, VIII, and IX

50
Q

Diagnostic Tool: measures platelet and vessel interaction, number of platelets, and function of platelets

A

Bleeding Time

51
Q

Diagnostic Tool: most commonly prolonged by vitamin K deficiency, liver disease, or warfarin/rat poison ingestion

52
Q

Diagnostic Tool: normally 9-12 seconds, but standardized to an international normalized ratio (INR) that is reported

53
Q

Diagnostic Tool: prolonged with heparin contamination, fibrinogen deficiency, or abnormal fibrinogen

A

Thrombin Time

54
Q

Diagnostic Tool: used to dianose Lupus anticoagulant

A

Russel’s Viper Venom Test (dRVVT)

55
Q

Diagnostic Tool: used to monitor heparin therapy

56
Q

Diagnostic Tool: used to monitor Warfarin therapy

57
Q

DIC shows a prolonged __ with less prolongation of the _____.

58
Q

Diganostic Tool: prolonged in Hemophilia A and B, XI or XII deficiency, Acquired hemophilia, von Willebrand Disease, Heparin, Lupus Anticoagulant

59
Q

Drugs: administered subcutaneously and has longer half life than heparin

A

Low Molecular Weight Heparins (LMWH) and Fondaparinux

60
Q

Drugs: antibodies to Platelet Factor 4/heparin complexes result in activation of platelets and a prothrombotic state

A

Heparin-Induced Thrombocytopenia/Thrombosis Syndrome (HIT)

61
Q

Drugs: anticoagulant with rapid absorption with good bioavailability and long half-life

62
Q

Drugs: binds to antithrombin III, and as a cofactor increases rate of thrombin inactivation by scale factor of 1000

63
Q

Drugs: complications associated with heparin therapy

A

Bleeding, Heparin-Induced Thrombocytopenia Syndrome (HIT), Allergic events

64
Q

Drugs: complications/contraindications of warfarin (3)

A

Hemorrhage, Pregnancy contraindication, food/drug interactions

65
Q

Drugs: direct thrombin inhibitors, used for patients with HIT

A

Argatroban (Novastan) and Lepirudin (Refludant)

66
Q

Drugs: fibrinolyitc, used for acute myocardial infarction, ischemic stroke, DVT, pulmonary embolism

A

t-PA, u-PA, streptokinase

67
Q

Drugs: full antithrombotic effect not achieved until existing coagulation factors in circulation are removed (3-5 days)

68
Q

Drugs: inhibitors block GPIIb/IIIa receptor and prevent platelet aggregation

A

Abciximab, Eptifibatide, Tirofiban

69
Q

Drugs: inhibits enzymes that use Vitamin K as a cofactor

70
Q

Drugs: occurs naturally in mast cell granules; extracted from porcine intestine for therapeutic use

71
Q

Drugs: oral antiplatelet agents that bind to ADP receptor on platelets, and block activation by ADP

A

Thienopyridines

72
Q

Drugs: oral ATP analogue that binds reversibly to ADP receptor

A

Ticagrelor

73
Q

Drugs: permanent damage to platelet (it cannot synthesize new proteins) via inhibition of cyclooxygenase

74
Q

Drugs: produced by chemical or enzymatic depolymerization of heparin to one-third the size of heparin

A

Low Molecular Weight Heparins (LMWH)

75
Q

Drugs: recombinant form of a leech anticoagulant

A

Lepirudin (Refludant)

76
Q

Drugs: selectively binds and inactivates factor Xa by antithrombin

A

Low Molecular Weight Heparins (LMWH) and Fondaparinux

77
Q

Drugs: small molecule direct thrombin inhibitor

A

Argatroban (Novastan)

78
Q

Drugs: synthetic pentasaccharide corresponding to minimal sequence required for heparin-like action

A

Fondaparinux

79
Q

Drugs: treatment and prevention of venous thrombosis, pulmonary embolism, acute myocardial infarction, surgery requiring bypass, etc.

80
Q

Extrinsic Tenase Factor Components

A

III, VIIa, IX or X, Ca and phospholipid

81
Q

Extrinsic Tenase Factor Products

A

IXa and Xa

82
Q

Factor Deficiency: autosomal recessive inheritance with greater incidence in Ashkenazi population, prolonged PTT

83
Q

Factor Deficiency: autosomal recessive, rare, prolonged PTT

84
Q

Factor Deficiency: Hemophilia A and B, level defined by amount of residual factor activity, X-linked

A

VIII, and IX

85
Q

Factor Deficiency: prolonged bleeding time and PTT, decreased Factor VIII

86
Q

Functions of von Willebrand Factor VWF

A

Carrier protein for Factor VIII, platelet adhesion

87
Q

In a one to one mixing study in a Lupus anticoagulant patient, the PTT is ______.

88
Q

In Lupus anticoagulant, the PTT can be resolved by adding _______.

A

excess phospholipid

89
Q

Intrinsic Tenase Factor Components

A

IXa, VIIIa, X, Ca and phospholipid

90
Q

Intrinsic Tenase Factor Products

91
Q

Lack of _______ leads to VIII deficiency since, _______ is a carrier for VIII

92
Q

Lack of ________ leads to abnormal platelet-endothelial interaction, leading to bleeding disorder of mucosal regions and skin

93
Q

Liver disease shows a prolonged with less prolongation of the _____.

94
Q

Lupus anticoagulant shows an ______ PTT

95
Q

Methods of Treating ITP

A

Steroids, IVIG, Splenectomy

96
Q

Mixing Studey (0, 2 hours): Normal, Normal

A

Hemophilia A

97
Q

Mixing Studey (0, 2 hours): Normal, Prolonged

A

Acquired Factor VIII Antibody

98
Q

Mixing Studey (0, 2 hours): Prolonged, Prolonged

A

Lupus Anticoagulant

99
Q

Name all factors activated by thrombin.

A

XI, IX, VIII, V, XIII, and I

100
Q

Neonatal purpura fulminans: homozygote for _________.

A

Protein S or Protein C

101
Q

Platelet _____ granules acid hydrolases

102
Q

Platelet ______ granules: proteins, especially fibrinogen, V, von Willebrand factor, as well as platelet activation factors and platelet growth factors

103
Q

Platelet integrin _______ increases affinity for vWF

A

GPIIb-IIIa

104
Q

Point mutation in Factor V that changes glutamine to arginine

105
Q

Prothrombinase Components

A

Xa, Va, II, Ca and phospholipid

106
Q

Prothrombinase Products

107
Q

PT measures the _______ pathway.

108
Q

PTT/APTT measures the _______ pathway.

109
Q

Rate of activation of X to Xa is 50-100 times faster by the _____ tenase complex.

110
Q

Serine Protease Factors

A

Prekallikrein, XII, XI, IX, X, VII, II

111
Q

TFPI can also bind ____ (less affinity) to form a TFPI-____-TF-VIIa complex

112
Q

TFPI can bind to ____ at its active site, and the ____ complex inactivates _____.

A

Xa; TFPI-Xa; TF-VIIa

113
Q

Thrombin activates ________ in the amplification phase of clotting.

A

V and VIII

114
Q

Thrombin binds to thrombomodulin on endothelial cells, creating a complex that can bind _______.

115
Q

Thrombin cleaves off _________ and _________, exposing sites on E domain that aligns complimentarily with D domain on another fibrin molecule

A

Fibrinopeptide A, Fibrinopeptide B

116
Q

Treatment of vWF deficiency

A

Arginine vasopressin, Factor Replacement

117
Q

Vitamin K dependent Factors

A

II, VII, IX, and X (Protein C and S)

118
Q

VWD Stage __: abnormal clearance by platelets

119
Q

VWD Stage __: partial quantitative deficiency

120
Q

VWD Stage __: qualitative deficiency

121
Q

VWD Stage __: severe or total quantitative deficiency

122
Q

XIIIa can covalently link _______ to fibrin to stabilize the clot

A

α2-antiplasmin

Blood and Lymph (14 decks)

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