Secondary Hemostasis Flashcards
1
Q
Where is coagulation factors synthesized?
A
Liver
2
Q
These factors are identified by Roman numerals based on the order of their discovery?
A
International Committee for the Standardization of the Nomenclature of the Blood Clotting Factors
3
Q
What does the ‘a’ denote in a coagulation factor?
A
Activated
4
Q
This is factor I and II?
A
Fibrinogen (I) and Prothrombin (II)
5
Q
This is a used crude mixture of tissue and phosoholipid, also called thromboplastin?
A
Tissue Factor (III)
6
Q
This factor is often referred to by its name or chemical symbol, not by its numeral?
A
Calcium (IV)
7
Q
This factor is later determined to be an activated factor V, but was withdrawn from the naming system and was never reassigned?
A
Assigned Procoagulant (VI)
8
Q
This is a cofactor that circulates linked to a large carrier protein, much like VWF?
A
Antihemophilic Factor (VIII)
9
Q
Also called as the Fletcher factor and belong to the kallikrein system?
A
Prekallikrein (Pre-K)
10
Q
This factor never received a roman numeral and primarily functions as a lie within the system?
A
Prekallikrein (Pre-K) and Highmolecular-Weight Kininogen (HMWK)
11
Q
Also called as the Fitzgerald factor and belong to the kinin systems?
A
Highmolecular-Weight Kininogen (HMWK)
12
Q
This is collectively called as platelet factor 3?
A
Platelet Phospholipids
13
Q
What is the specific platelet phospholipid being described in factor III?
A
Phosphatidylserine (III)
14
Q
This is collectively called the 8 inactive enzymes that circulate the coagulation system?
A
Zymogen
15
Q
These compounds bind, stabilize, and enhance the activity of their respective enzymes like serine proteases?
A
Cofactors
16
Q
This compound acts as a substrate for the enzymatic action of thrombin?
A
Fibrinogen
17
Q
This is the primary enzyme for the coagulation system?
A
Thrombin
18
Q
These act as control proteins responsible for regulating the coagulation process to avoid unnecessary blood clotting?
A
Glycoproteins
19
Q
These are the co-factors involved in the coagulation system?
A
HMWK
Tissue Factor
Factor 5, 8
Protein Z, S
Thrombomodulin
20
Q
These are the zymogens involved in the coagulation system?
A
Pre-Kallikrein
Prothrombin
Factor 7, 9, 10, 11, 12, 13
21
Q
These are the control proteins involved in the coagulation system?
A
Anti-thrombin
Heparin Cofactor 2
Anti-Tissue Factor Pathway Inhibitor (TFPI)
A2-Macroglobulin
A1-Antitrypsin
Protein Z-Dependent Protease Inhibitor (ZPI)
22
Q
These are the classifications of procoagulants?
A
Serine Protease
Cofactors
Tranglutaminases
23
Q
This coagulant factor functions as a transglutamase and transamidase?
A
Factor XIII
24
Q
These are proteolytic enzymes that cleave other proteins to propagate the clotting cascade?
A
Serine Proteases
25
What are the serine proteases in the coagulation system?
Activated Factor 2, 7, 9, 10, 11, 12
Pre-Kallikrein
26
What are the cofactors in the coaglation system?
Tissue Factors
Factor 5, 8
HMWK
27
This is the ultimate substrate of the coagulation pathway and forms the primary structural protein of the fibrin clot?
Fibrinogen
28
What hydrolyzes fibrinogen and what stabilizes the fibrin clot?
Thrombin hydrolyzes the fibrinogen, forming a clot which is stabilized by Factor 13.
29
This plays a crucial role in assembling coagulation complexes on platelet membranes?
Calcium
30
An uncontrolled activated of calcium and fibrinogen often leads to what condition?
Disseminated Intravascular Coagulation (DIC)
31
These are the procoagulants and regulatory proteins that are vitamin k-dependent?
Prothorombin
Factor 7, 9, 10
Protein C, S, Z
32
These are the common features found in the vitamin k-dependent regulatory proteins?
10 to 12 glutamic acids units near amino termini
33
This is the principle of vitamin k?
Catalysis of y-carboxlation of glutamic acids then binds to calcium.
34
Deficiency in vitamin k often leads to the production of what compound?
Proteins Induced by Vitamin K Absence or Antagonists (PIVKAs)
des-y-carboxyl proteins
35
This compound acts as a vitamin k antagonists?
Warfarin
36
This is the principle of Warfarin?
Vitamin K Expoxide Reductase Inhibition
37
This is responsible for recylcing vitamin k in its active form?
Vitamin K Exposide Reductase
38
These proteins are unable to effectively bind calcium or phospholipids, which are crucial for
their proper function in the coagulation cascade?
PIVKAs
39
This is the acquired condition/consequence of vitamin k deficiency or warfarin?
Increased risk of bleeding and impaired blood clot.
40
These are the complexes formed during the coagulation cascade?
Extrinsic, Intrinsic, Prothrombinase
41
This is the composition of extrinsic complexes and the corresponding factors they activate?
Factor 7a, Tissue Factor
Activates Factor 9 (minor), 10
42
This is the composition of intrinsic complexes and the corresponding factors they activate?
Factor 8a, 9a
Activates 10
43
This is the composition of prothrombinase complexes and the corresponding factors they activate?
Factor 5a, 10a
Converted into Prothrombin
44
This is used as an anticoagulat via vitamin k inhibition?
Warfarin
45
What is usually being monitored in warfarin therapy?
PIVKAs and F1.2
46
In warfarin therapy, what is used in cases of deficiency to restore normal coagulation?
Vitamin K Supplementation
47
This procoagulant functions as a transmembrane receptor for factor 7a and initiates coagulation?
Tissue Factor
48
This is located in extravascular cells and increases in expression via leukocytes during inflammation and sepsis?
Tissue Factor
49
This procoagulant functions as a cofactor to 10a in the prothrombinase complex?
Factor 5
50
This procoagulant is located in plasma and platelet a-granules, released during platelet activation?
Factor 5
51
What inactivates factor 5?
Protein C to Vi
52
This procoagulant functions as a cofactor to factor 9 in the intrinsic tenase complex and is regulated by thrombin?
Factor 8
53
This procoagulant functions as a cofactor to factor 12a and Prekallikrein?
High-Molecular-Weight Kininogen
54
This procoagulant is part of the intrinsic contact factor complex as is activated in the presence of foreign objects or inflammation?
High-Molecular-Weight Kininogen
55
This control cofactor functions as thrombin cofactor and regulates coagulation and fibrinolysis?
Thrombomodulin
56
What compounds does thrombomodulin activates and suppresses?
Activates Protein C and TAFI
Suppresses Factor 5 and 8
57
This control cofactor functions as a cofactor to protein C and TFPI; it also acts as regulator for coagulation?
Protein S
58
This is the cofactor to Z-dependent potease inhibitor and act as assistance to coagulation control?
Protein Z
59
The examples of these types of cells are fibroblasts and smooth muscle cells?
Extravascular Cells
60
This factor has a molecular mass of 260k Daltons, made by hepatocytes and microvascular EC's, bound or unbound to VWF, and is cleaved and activated by thrombin?
Factor 8
61
Factor 8 is found in the intrinsic tenase complex to bind with?
Factor 9a and Ca2
62
This inactivates factor 8?
Protein C
63
Factor 8 is what type of linked trait and where is it relevant in?
X-linked Male Trait and Relevant in Hemophilia A
64
This condition has a deficient factor 8 but normal VWF level?
Hemophilia A
65
The blood with this condition is hard to store due to its rapid deterioration and drop when thawed?
Hemophila A
66
How is hemophilia A treated?
12-hour Half-life Factor 8 Replacement Therapy
67
This is a large multimeric glycoprotein with 240k daltons that participates in adhesion and transport of factor 8?
Von Willebrand Factor
68
These are the cells that produce and store VWF?
Made in ECs, Megakaryocytes
Stored in a-granules and Weibel-Palade bodies
69
These are the binding sites of the VWF?
Platelets (gp 1b/2a and gp 2b/3a)
Collagens
Factor 8
70
The function of the VWF is especially cruical in fast blood flow areas like?
Arteries and Arterioles
71
These are activated through contact with negatively charged surfaces?
Factor 12, HMWK, and Pre-K
72
This factor muste be activated in order to converts Pre-K into Kallikrein?
Factor 12
73
This contact factor is responsible for cleaving HMWK into bradykinin?
Kallikrein
74
This factor is initiated through the contact factor complex (Pre-K and HMWK) and Thrombin?
Factor 11
75
This is activated due to the activation of Factor 11, contributing to the instrinsic pathway?
Factor 9
76
This is the consequence of being deficient in contact factors (HMWK, Pre-K, and Factor 12)?
Prolonged lab tests, but no bleeding
77
What is required in order to activate factor 12 in-vitro?
Negative Surface Charge
78
This is a condition described as having a mild and variable bleeding due to factor 11 deficiency?
Rosenthal Syndrome
79
Why is the Rosenthal Syndome generally less severe than deficiencies of Factor 8 or 9?
Factor 11 supplements Factor 9 activation.
80
This is the consequence of in-vivo activation via stents, prosthesis, and other foreign materials?
Thrombosis Risk
81
This compound cleaves fibrinopeptides A and B from fibrinigen to form fibrin?
Thrombin
82
Thrombin amplifies coagulation via activation of what cofactors?
Cofactor 5, 8, and 11
83
What is initiated by thrombin?
Platelet Aggregation Initiation
84
This pathway suppresses coagulation and the other suppresses fibrinolysis
C Pathway (Suppress Coagulation)
TAFI (Suppress Fibrinolysis)
85
This is the clinical significance of thrombin?
Target for anticoagulantion therapy
86
This factor is 340k Dalton, 200-400 mg/dL plasma level, with a trinodular dimer of Aa, Bb, and g polypeptides?
Fibrinogen
87
This factor links platelets via go 2b/3a receptors?
Fibrinogen
88
What are two subunits of factor 13 and where are they produced?
Subunit A in Mega/Monocytes
Subunit B in Liver
89
This factor cross-links fibrin polymers via covalent bonds for stability and is essential in wound healing and tissue integrity?
Factor 13
90
This is the fibrinogen domain that is composed of 6 N-terminals that forms a bulk region?
E Domain
91
This fibrinogen domain is made up of 3 carboxyl terminals on each outer end?
D Domain
92
This compound cleaves fibrinogen via hydrolysis into two subunits?
Thrombin
93
This is the principle on why cleaved fibrinogens form a fibrin polymer?
E domain and D domain Affinity
94
This control protein is involved in cell adhesion and covalently incorporated in the cross-linking of polymers by factor 13a?
Fibronectin
95
This control protein is involved in factor 13, rendering the fibrin meshwork resistant to fibrinolysis
a2-antiplasmin
96
This is the primary serine protease in the fibrinolytic system?
Plasminogen
97
This covalently bounds plasminogen to fibrinolytic system?
Lysine Moieties
(Lysine Amino Acid)
98
This is a serine protease that ultimately hydrolyses and activates bound plasminogen?
Tissue Plasminogen Activator
99
This is the clinical significance of fibrinogen?
Acute Phase Reactant
100
This is the clinical significance of Factor 13?
Less wound healing and bleeding disorders
101
This is described as the sequential activation of enzymes leading to clot formation?
Cascade Model
102
This is the factor that is activated and regarded by experts as the primary step in coagulation because it is found in blood, unlike tissue factor (III)?
Activation of Factor 12
103
What are the key factors involved in the intrinsic pathway?
Pre-K, HMWK
Factor 1, 2, 5, 8, 9, 10
104
What laboratory test evaluates the intrinsic pathway?
Activated Partial Thromboplastin Time (APTT/PTT)
105
Why is the intrinsic pathway more relevant in laboratory testing than in trauma-related coagulation?
Assessed in vitro, not much in vivo
106
What are the key coagulation factors involved in the extrinsic pathway?
Factor 1, 2, 3, 5, 10, 7
107
How is the extrinsic pathway initiated?
Formation of the TF complex (TF:VIIa)
108
What laboratory test evaluates the extrinsic pathway?
Prothrombin Time (PT)
109
What is the primary in vivo initiation mechanism for coagulation?
The tissue factor (TF) pathway
110
What are the key coagulation factors involved in the common pathway?
Factors 1, 2, 5, 10
111
What is the end result of the common pathway?
Generation of thrombin and formation of a fibrin clot.
112
Why is the common pathway important in laboratory testing?
Both PT and PTT Tests
113
What coagulation factors are deficient in Hemophilia A and B?
Hemophilia A: Factor VIII deficiency
Hemophilia B: Factor IX deficiency
114
How do Hemophilia A and B affect the coagulation pathway?
They affect the intrinsic pathway
115
What laboratory tests are used to diagnose specific factor deficiencies?
PT and PTT
116
What are the clinical implications of factor deficiencies in hemophilia?
Severe bleeding risks
117
What are the limitations of the traditional extrinsic and intrinsic pathway models?
Traditional models in vivo these
Each pathways are interdependent.
118
What is the role of tissue factor-expressing cells in coagulation?
Extravascular and initiate coagulation
119
What is the function of platelets in coagulation?
Intravascular Cells for Propagation
120
Why is the interaction between tissue factor-expressing cells and platelets important?
Effective coagulation
121
What is the key process in the initiation phase of coagulation?
Formation of the extrinsic tenase complex on tissue factor-bearing cells
122
How much thrombin is generated during the initiation phase?
Small amounts, about 3-5% of total thrombin
123
What is the role of Factor VIIa in the initiation phase?
It binds to tissue factor and activates Factor IX and Factor X
124
Why is the initiation phase important in coagulation?
It sets the stage for large-scale thrombin production in the propagation phase.
125
What initiates platelet activation in the initiation phase of coagulation?
Low levels of thrombin
126
Which clotting factors are activated during the initiation phase of coagulation?
Factors V, VIII, and XI
127
What is the role of the preliminary fibrin network in coagulation?
Establishes an initial platelet plug
128
What marks the transition to the propagation phase in coagulation assays?
Fibrinopeptide cleavage
129
Key process in the propagation phase of coagulation?
Thrombin amplification on platelets
130
Role of platelets in the propagation phase?
Platform for intrinsic tenase and prothrombinase complex
131
What percentage of thrombin is generated in the propagation phase?
95%
132
What factor is responsible for clot stabilization?
Factor 13
133
Where does coagulation occur in vivo?
At the site of injury
134
What prevents widespread clotting?
Protease inhibitors
135
How is blood flow maintained in non-injured areas?
Endothelial protection
136
What is the main regulator of the tissue factor pathway?
Tissue Factor Pathway Inhibitor (TFPI)
137
What does Antithrombin (AT) inhibit?
Thrombin and other serine proteases.
138
What is the function of Activated Protein C (APC)
It inactivates factors Va and VIIIa
End of Protein C Pathway
139
What type of inhibitor is Tissue Factor Pathway Inhibitor (TFPI)?
A Kunitz-type serine protease inhibitor
140
What are the two steps of TFPI function?
Binds and inactivates factor Xa
TFPI:Xa complex binds to TF:VIIa,
= No activation of factors X and IX
141
How does Protein S influence TFPI activity?
Protein S enhances TFPI’s inhibition of factor Xa
142
What is the regulatory importance of TFPI?
TFPI ensures that extrinsic tenase activity is short-lived
143
What dual role does thrombin play?
Procoagulant and an Anticoagulant
144
What is the function of the Protein C regulatory system in intact vessels?
Inhibits excessive coagulation
145
What initiates the activation of the Protein C regulatory system?
Thrombin binds to thrombomodulin
146
What happens after Protein C is activated to Activated Protein C (APC)?
Binds to Endothelial Protein C Receptor (EPCR)
147
What role does free plasma Protein S play in the Protein C system?
APC binds to Protein S, forming a stabilized complex
148
How does the APC-Protein S complex regulate coagulation?
Hydrolyzes and inactivates factors Va and VIIIa
149
What can deficiencies in Protein C or Protein S lead to?
Thrombotic disorders
150
What types of deficiencies can cause Protein C system dysfunction?
Acquired or inherited deficiencies (Mutations)
151
What severe condition can result from a lack of Protein C in neonates?
Purpura fulminans, a severe thrombotic condition
152
A family of proteins that regulate serine proteases?
Serpins
153
Serpins involved in coagulation regulation?
Antithrombin (AT)
Heparin Factor 2
Protein Z-Dependent Protease Inhibitor (ZPI)
Protein C Inhibitor
a1-Protease Inhibitor
a2-Macroglobulin
a2-antiplasmin
Plasminogen Activator Inhibitor-1 (PAI-1)
154
It inhibits serine proteases including thrombin, factors 9a, 10a, 11a, 12a, prekallikrein, and plasmin?
Antithrombin (AT)
155
What is required for Antithrombin (AT) activation?
Heparin
156
Accelerates the inactivation of thrombin and other clotting factors by Antithrombin (AT) up to 2000-fold?
Heparin
157
What is the primary function of Heparin Cofactor II?
Inactivates thrombin
158
Heparin made of long chains; accelerates thrombin inactivation?
Unfractioned Heparin
159
Heparin that preferentially inactivate factor 10a
Low-Molecular-Weight Heparin and Pentasaccharide Heparins
160
A complex formed by ntithrombin covalently binding thrombin?
Thrombin-Antithrombin Complex (TAT)
161
This serves as an indicator of thrombosis, reflecting thrombin
generation and inhibition.
Thrombin-Antithrombin Complex (TAT)
162
A potent inhibitor of factor 10a and factor 11a
Protein Z-Dependent Protease Inhibitor (ZPI)
163
Enhances ZPI’s inhibition ability 2000-fold
Protein Z
164
It acts as an anticoagulant, procoagulant, or fibrinolytic inhibitor depending on the target?
Protein C
165
Inhibits APC, thrombin, factor 10a, factor 11a, and urokinase?
Protein C Inhibitor
166
It reversibly inhibits serine proteases?
a1-Protease Inhibitor (a1-Antitrypsin)
a2-Macroglobulin
167
What is the role of α2-Antiplasmin and Plasminogen Activator Inhibitor-1?
Regulate Fibrinolysis
168
What are the sources of vitamin K?
Green Leafy Vegetables
Escherichia coli
Bacteroides fragilis
