hema cram Flashcards

1
Q

What is the physiologic response to vascular injury?

A

Limit blood loss

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2
Q

What are the characteristics of hemostasis?

A

Rapid, localized, well-regulated

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3
Q

Who published a sequence of proteolytic reactions for coagulation?

A

MacFarland

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4
Q

What begins the coagulation cascade?

A

Factor XII activation

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5
Q

What substance does Thrombin proteolyze to form a clot?

A

Fibrinogen

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6
Q

What did patients with Factor XII Deficiency show?

A

Did not bleed

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7
Q

What is a key feature of the cell-based model of hemostasis?

A

Cellular elements express TF

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8
Q

Which cells provide a phospholipid surface for coagulation?

A

Endothelium, Platelets

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9
Q

What does Thrombin convert Fibrinogen into?

A

Fibrin Clot

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10
Q

What are the two groups of zymogens in the Coagulation System?

A

Phospholipid-Bound, Surface-Bound

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11
Q

What is the role of Vitamin K in coagulation proteins?

A

Essential for γ-carboxylation

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12
Q

What does the γ-carboxylation reaction allow coagulation proteins to do?

A

Bind to phospholipid and cell membranes

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13
Q

What is necessary for proteins FX, FIX, FVII, and FII to function normally?

A

Carboxylation reaction

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14
Q

What does activated Protein C do?

A

Inactivates FVa and FVIIIa

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15
Q

What role does Protein S play?

A

Natural anticoagulant

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16
Q

What is K-dependent and acts as cofactor for APC?

A

Surface-Bound

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17
Q

What are the proenzymes of the Plasma Kallikrein/Kinin System?

A

FXII, Prekallikrein, FXI

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18
Q

What are the protein zymogens of the CONTACT SYSTEM?

A

FXII autoactivation

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19
Q

What common laboratory test uses FXII autoactivation?

A

Activated Partial Thromboplastin Time (APTT)

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20
Q

What are the types of Urokinase Plasminogen Activator?

A

ScuPA, TcuPA

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21
Q

Where are these plasminogen activators found?

A

Endothelium, Neutrophils, Monocytes

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22
Q

What do tPA, ScuPA, and TcuPA do?

A

Convert Plasminogen to Plasmin

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23
Q

How is tPA produced?

A

Constitutively

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24
Q

What increases ScuPA levels?

A

Inflammatory states

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25
Q

What is the major inhibitor of tPA and TcuPA?

A

Plasminogen Activator Inhibitor-1 (PAI-1)

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26
Q

What can TAFI do to control Plasmin?

A

Remove lysine residues from Fibrin

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27
Q

What is the active Plasmin molecule?

A

A potent protease

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28
Q

What does Plasmin do to soluble Fibrinogen?

A

Produces Fibrinogen Degradation Products

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29
Q

What does Plasmin cleave Fibrinogen into?

A

An X Fragment

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30
Q

What complex does TFPI form?

A

FVIIa, TF, FXa

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31
Q

Where is TFPI produced?

A

Microvascular endothelium

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32
Q

What condition is associated with murine TFPI knockout?

A

Embryonically lethal

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33
Q

Where is AβPP present?

A

Platelets and brain

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34
Q

Which factors does AβPP regulate?

A

XIa, IXa, Xa, VIIa/TF

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35
Q

What does AβPP not inhibit?

A

Thrombin

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36
Q

What enzyme produces Prostacyclins (PGI2)?

A

Cyclooxygenase-2

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37
Q

What is the function of PGI2?

A

Vasodilator, inhibits platelet aggregation

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38
Q

What hormone is involved in controlling hemostasis?

A

Vasoactive hormones

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39
Q

What are believed to be cerebral anticoagulants?

A

Prostacyclins, Nitric Oxide, Thrombospondin-5

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40
Q

How do Prostacyclins mediate their actions?

A

IP Receptors and PPARs

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41
Q

What enzyme do endothelial cells express?

A

eNOS (NOS3)

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42
Q

What is a significant function of Nitric Oxide?

A

Potent vasodilator

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43
Q

What does NO inhibit?

A

Platelet adhesion and aggregation

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44
Q

What does NO activate?

A

Guanylate Cyclase and cGMP

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45
Q

What do activated platelets produce under shear flow?

A

NO

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46
Q

What is Thrombospondin-5 also known as?

A

COMP

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47
Q

What type of protein is COMP?

A

Extracellular protein

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48
Q

What tissues release COMP?

A

Cartilage and muscle

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49
Q

Common presentations of Secondary Hemostasis defects?

A

Ecchymoses, soft tissue, joint bleeds

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50
Q

Types of bleeding tendencies?

A

Congenital or acquired

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51
Q

What is necessary before laboratory testing for bleeding?

A

Personal and family history

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52
Q

When are congenital bleeding disorders usually diagnosed?

A

Early childhood

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53
Q

When might mild bleeding disorders be revealed?

A

Later in life with hemostatic challenge

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54
Q

What are Routine Coagulation Screening Tests used for?

A

Identify hemostatic defects

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55
Q

Is it clinically useful to perform coagulation tests on nonbleeding patients?

A

Not clinically useful

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56
Q

What tests are considered screening coagulation tests?

A

PT, APTT, Fibrinogen, Thrombin Time, D-Dimers

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57
Q

What is the collection ratio for whole blood in Sodium Citrate?

A

9:1 Blood:Citrate

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58
Q

How should blood specimens be mixed after collection?

A

Gently invert 3 to 5 times

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59
Q

What is the transport requirement for the specimen?

A

Room temperature, within 4 hours

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60
Q

What should be done if testing cannot be performed within 4 hours?

A

Centrifuge and freeze

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61
Q

Why should separated plasma be platelet free before freezing?

A

To prevent false low anti-FXa

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62
Q

What is the threshold for platelet count in separated plasma before freezing?

A

<10 K

63
Q

What does platelet factor 4 bind to?

A

Heparin

64
Q

What is the Fibrin Stabilizing Factor?

A

XIII

65
Q

Cellular Component consists mostly of what?

A

PLATELETS and ENDOTHELIAL CELLS

66
Q

What does the Large Group of Plasma Proteins participate in?

A

clot formation

67
Q

What is vascular integrity BREACHED?

A

vascular injury

68
Q

Where is it found?

A

most tissues and cells

69
Q

What is the cause of the formation of complexes with FVII?

A

hemostatic reactions

70
Q

What results in the formation of complexes with FVII that produce the initiation of hemostatic reactions?

A

Upregulation of TF

71
Q

What is the cofactor for FIXa when it is activated?

A

FVIIIa

72
Q

What is the most severe clinically recognized bleeding disorder?

A

Hemophilia A. FV

73
Q

What is a protein with homology to FVIII?

A

330-kDa

74
Q

What is the main substrate of FII?

A

Thrombin Fibrinogen

75
Q

What is the principal adhesive molecule for platelet aggregation?

A

330-kDa

76
Q

What proteolyzes Fibrinogen?

A

Thrombin

77
Q

What is formed when Fibrinogen is proteolyzed by Thrombin?

A

a FIBRIN MONOMER

78
Q

What type of FIBRIN CLOT do monomers associate end to end and side to side to form?

A

polymerized FIBRIN CLOT

79
Q

What are the most prominent bleeding disorders that occur in patients who survive gestation and birth?

A

Deficiencies in coagulation Factors VIII and IX

80
Q

What type of patients have congenital deficiencies of coagulation Factor VII, X, V, and II?

A

rare

81
Q

Rare patients who have what type of deficiencies of coagulation Factor VII, X, V, and II usually do not have severe bleeding states?

A

congenital deficiencies

82
Q

What is the order structure of the Prothrombinase Complex?

A

FII (Prothrombin)

83
Q

What are the components of the Prothrombinase Complex?

A

phospholipid membranes or cell membranes

84
Q

What is the Endothelial Protein C Receptor?

A

EPCR

85
Q

What does the complex formed by APC+EPCR need to do to allow binding of APC with Protein S to inactivate FVa and

A

DISSOCIATE

86
Q

How does APC bind to cell surfaces in a way that orients itself to inactivate?

A

FVa and FVIIIa

87
Q

What does the enzyme use to localize its activity to perform its inhibitory function?

A

receptor

88
Q

What is in equilibrium between the FREE FORM (40%) and a BOUND FORM (60%)?

A

Plasma Protein S

89
Q

How many Receptors on Endothelial Cells bind APC to?

A

3

90
Q

What is the PAR?

A

Protease-Activated Receptor

91
Q

What may contribute to the anticoagulant function of Activated Protein C by liberating tPA?

A

Activation of PAR1

92
Q

What is the inhibitor effect on FIIa Heparin Cofactor II?

A

4000-fold

93
Q

What is Factor Xa dependent?

A

Vitamin K3

94
Q

What is the causative factor for Types I and II Hereditary Angioedema?

A

The absence of C1 Inhibitor

95
Q

What is the most potent inhibitor of the FVIIa-Tissue Factor Vomplex?

A

Kunitz-Type Serine Protease Inhibitor

96
Q

Who was the downloader of lOMoARcPSD?

A

Princess Therese Timbal

97
Q

What does COMP inhibit?

A

Thrombin

98
Q

What are EXPOSED at the site of endothelial cell injury?

A

Subendothelial Collagen and Von Willebrand Factor

99
Q

What does the platelet undergo from DISCOID to SPHERICAL?

A

SHAPE CHANGE

100
Q

GPIb/IX/V Receptors are binding to what?

A

vWF

101
Q

What is followed by platelet Adhesion?

A

SECRETION

102
Q

What is the name of the path that releases the contents of Alpha and Dense Granules from Platelets?

A

the Cyclooxygenase-1 (COX-1) Pathway

103
Q

What parts of Platelets are released during Secretion?

A

Alpha and Dense Granules

104
Q

What causes ACTIVATION TXA2?

A

vasoconstriction

105
Q

ACTIVATION TXA2 causes vasoconstriction and ACTIVATES PLATELETS via what?

A

TXA receptor

106
Q

What leads to exposure of Phosphatidylserine?

A

Activation of Platelets

107
Q

What does Phosphatidylserine begin to produce?

A

Thrombin

108
Q

What can be divided into 2 different phases?

A

secondary Hemostasis

109
Q

TF PATHWAY is the initiator of what?

A

coagulation 2.

110
Q

What is the name of TF PATHWAY?

A

EXTRINSIC PATHWAY

111
Q

INTRINSIC PATHWAY amplifies and propagates what?

A

thrombin generation

112
Q

What does the TF Pathway begin with on the cell surface?

A

ENDOTHELIAL INJURY and EXPRESSION OF TF

113
Q

What is the name of the complex that activates FX to FXa?

A

Extrinsic Tenase

114
Q

Extrinsic Tenase activates FX to FXa on the cell surface in the presence of what?

A

Ca++

115
Q

What does ASE COMPLEX form?

A

Thrombin

116
Q

What is the cell surface of the FXa?

A

PL

117
Q

What converts Prothrombin to Thrombin?

A

PROTHROMBINASE COMPLEX

118
Q

What is the cell surface of the Intrinsic Pathway?

A

Ca++

119
Q

What is the cell surface of FIXa?

A

PL

120
Q

What forms the INTRINSIC TENASE?

A

FIXa

121
Q

What is the primary pathway of THROMBIN FORMATION?

A

Intrinsic Pathway

122
Q

What is the Intrinsic Pathway?

A

AMPLIFIER AND PROPAGATOR of Thrombin

123
Q

What is the INITIATOR?

A

TF Pathway

124
Q

What would patients with Hemophilia A and B have if they were independent pathways producing Thrombin?

A

bleeding tendencies

125
Q

What are the test tubes that assess Thrombin?

A

Prothrombin Time (PT) and Activated Partial Thromboplastin Time (APTT)

126
Q

What is the main enzyme in the coagulation system?

A

Thrombin

127
Q

What does Thrombin convert Fibrinogen to?

A

Fibrin Monomers

128
Q

What does it activate to cross-link fibrin monomers to stabilize the clot?

A

FXIII to FXIIIa

129
Q

What are released from platelet membranes?

A

Phospholipids

130
Q

What do Phospholipids release from platelet membranes bind to?

A

Lupus Anticoagulant

131
Q

How long should the specimen be held at room temperature?

A

4 hours

132
Q

What is often encountered in office clinics that send out their specimens to remote reference laboratories for coagulation testing?

A

Improper sample handling

133
Q

What part of coagulation testing is crucial for accurate diagnosis and management?

A

preanalytic

134
Q

What does PT contain?

A

tissue Thromboplastin and CaCl2

135
Q

What are the Coagulation Factors in the Intrinsic Pathway and what other path?

A

Common Pathway

136
Q

What does APTT generally assess in the Intrinsic Pathway and Common Pathway?

A

Coagulation Factors

137
Q

What type of contact factor is activated in APTT?

A

PLs

138
Q

What is the activation of in APTT?

A

Contact Factors

139
Q

How many different quantities of PL are available in the APTT reagents?

A

three

140
Q

What are the three APTT reagents with different sensitivities for various needs?

A

a.

141
Q

What does APTT-FS have the highest amount of?

A

PL

142
Q

What is APTT-FS?

A

-Factor Sensitive

143
Q

What is the smallest amount of PL?

A

weak LA

144
Q

What does APTT-FSL detect?

A

decreased amounts of coagulation factors and lupus anticoagulant

145
Q

What is the term for PL?

A

c. APTT-FSL

146
Q

What is a common clinical finding?

A

prolonged APTT

147
Q

What should further investigation be dictated by if the presenting symptom is the presenting symptom?

A

a personal and family history of bleeding

148
Q

What is prolonged in a bleeding patient?

A

APTT

149
Q

What should be considered and investigated by measuring FVIII first?

A

a CONGENITAL BLEEDING DISORDER of INTRINSIC PATHWAY

150
Q

What is the name of the DISORDER of INTRINSIC PATHWAY?

A

FIX

151
Q

Why is Thrombin time not a commonly ordered test?

A

limited utility

152
Q

What is added to the patient’s plasma and the clotting time recorded?

A

Bovine Thrombin

153
Q

How long should the upper limit of the normal range be adjusted to make TT more sensitive to detect DYSFIBRINOGENEMIA?

A

25 sec