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Hematology 2 Lec > Coagulation Disorders > Flashcards

Coagulation Disorders Flashcards

1
Q

: proteins that initiate the IP

A

XII, HK, PK

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2
Q

denotes the time when plasma coagulation factors come in contact with tissue/artificial surfaces

A

CONTACT PHASE

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3
Q

product of contact phase:

A

IX

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4
Q

Not significant in the body when deficient because there are still intrinsic activators of FIX

A

XII, HK, PK

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5
Q

Presence or absence of these factors does not cause bleeding disorders because thrombin will still be generate independently = patient is asymptomatic = no manifestation of bleeding

A

XII, HK, PK

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6
Q

Tested via in vivo testing (not by mixing studies)

A

XII, HK, PK

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7
Q

The time it takes for free factors to activate is the time when the plasma will be adhering to tissue or artificial surfaces

A

CONTACT PHASE

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8
Q

CONTACT PHASE FACTORS DEFICIENCY
autosomal recessive disorder

A

Factor XII (Hageman factor) Deficiency

Prekallikrein (Fletcher factor) Deficiency

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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9
Q

clinical findings: no bleeding disorder but manifested by excessive clotting (but rather thrombotic disorder)

A

Factor XII (Hageman factor) Deficiency

PK

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10
Q

laboratory findings: all normal except APTT (prolonged)

A

Factor XII (Hageman factor) Deficiency

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11
Q

correction: adsorbed plasma, aged serum, fresh normal plasma (all reagent plasma with contact phase)

A

Factor XII (Hageman factor) Deficiency

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12
Q

treatment: none

A

Factor XII (Hageman factor) Deficiency

Prekallikrein (Fletcher factor) Deficiency

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13
Q

clinical findings: no bleeding disorder but manifested by excessive clotting

A

Prekallikrein (Fletcher factor) Deficiency

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14
Q

laboratory findings: all normal except APTT (shortened)

A

Prekallikrein (Fletcher factor) Deficiency

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15
Q

deficiency results to:
• poor contact phase reactions
• kinin formation deficiency
• defective fibrinolysis reaction

A

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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16
Q

APTT: normal to mildly prolonged (low deficiency)

A

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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17
Q

Thrombin will still be generated

A

Factor XII (Hageman factor) Deficiency

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18
Q

Thrombotic (abnormal formation of clots): FXII, aside from activating FXI, activates plasminogen to plasmin → more fibrinolysis

A

Factor XII (Hageman factor) Deficiency

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19
Q

CF stops the bleeding during injury → Coagulation cascade continues to activate = ↓ FXII = ↓ Plasmin = ↓Fibrinolysis = ↑ Clot formation = Thrombosis

A

Factor XII (Hageman factor) Deficiency

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20
Q

even if it has nothing to do w/ bleeding disorders

A

Factor XII (Hageman factor) Deficiency

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21
Q

Does not cause bleeding disorders; Asymptomatic

A

Factor XII (Hageman factor) Deficiency

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22
Q

FXII will bring (?) (cofactor) to activate plasminogen to plasmin

A

Prekallikrein (Fletcher factor) Deficiency

Kallikrein

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23
Q

↓ Kallikrein = (?) Clot formation = (?)

A

Prekallikrein (Fletcher factor) Deficiency

↑

Thrombosis

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24
Q

(?) APTT: PK reacts with (?) = (?) contact phase activation; indicates (?) of PK

A

Prekallikrein (Fletcher factor) Deficiency

shortened

kaolin (activator)

speeds up

low deficiency

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25
Q

(?) APTT: indicates a (?) of PK in the circulation

A

Prekallikrein (Fletcher factor) Deficiency

prolonged

severe deficiency or absence

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26
Q

Does not cause bleeding disorders

A

FXII

Prekallikrein (Fletcher factor) Deficiency

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27
Q

Absence = Lacks cofactor

A

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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28
Q

Inability to produce bradykinin (↓ inflammatory responses)

A

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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29
Q

can also be a cofactor of FXII and K with activating plasminogen to plasmin

A

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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30
Q

Prolonged: severe deficiency

A

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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31
Q

Not significant in vitro; No haemorrhagic tendency

A

HMWK (Fitzgerald/Flaujeac factor) Deficiency

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32
Q

complete absence or total deficiency of the entire HWMK

A

Fitzgerald

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33
Q

presence of HWMK, but absence of kinogen (kinogen: deficient protein portion)

A

Flaujeac (William’s Trait)

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34
Q

“love of hemorrhage”

A

Hemophilias

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35
Q

are inherited bleeding disorders characterized by a deficiency of the antihemophilic factors.

A

Hemophilias

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36
Q

<1%

A

Severe

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37
Q

Spontaneous bleeding

A

Severe

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38
Q

1-5%

A

Moderate

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39
Q

Spontaneous bleeding is uncommon

A

Moderate

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40
Q

may bleed with surgery or trauma

A

Moderate

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41
Q

5-20%

A

Mild

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42
Q

No spontaneous bleeding

A

Mild

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43
Q

may bleed with major surgery or trauma

A

Mild

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44
Q

CONTACT PHASE FACTORS DEFICIENCY :

A
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45
Q

INHERITED INTRINSIC PATHWAY HEMORRHAGIC DISORDERS :

A
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46
Q

most common hemophilia

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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47
Q

sex-linked disorder transmitted on the X chromosome by carrier women to their sons (50% affected)

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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48
Q

activation of VIII:C: thrombin (IIa)

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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49
Q

role: cofactor to FIX to form intrinsic tenase in activation of FX

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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50
Q

clinical signs and symptoms:
• severe GIT and intracranial hemorrhage
• prone to hemarthrosis

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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51
Q

laboratory findings: prolonged APTT

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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52
Q

correction: aged plasma, fresh adsorbed plasma (any plasma w/ FVIII preent)

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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53
Q

not corrected by: fresh serum (consumed during coagulation) or aged serum

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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54
Q

treatment/therapy (same In VW because it sirculates in complex with VIII:C):
• cryoprecipitate: to arrest bleeding
• Prophylactic DDAVP (I-desamino-8-D arginine-vasopressin)

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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55
Q

exist in the royal family (Queen Victoria); incest: sharing of genes

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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56
Q

mother always carry a carrier gene; affected X chromosome carries Hemophilia A (H-A) or a defect in Factor VIII:C

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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57
Q

normal distribution:
o male: carries X from mother and Y from father
o female: carries X from mother and X from father

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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58
Q

in H-A:
o X from mother can be passed to male offspring (higher chance of inheriting the trait due to one X chromosome coming from the mother only)
o male linkage of the family: affected by H-A

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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59
Q

activators of VIII:C:

A

residual thrombin, low level thrombin from the cascade

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60
Q

(blood trapped in the joints)

A

hemarthrosis

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61
Q

crippling and deformed hip, elbow, ankle, and shoulder due to continuous hemarthrosis

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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62
Q

o used to differentiate hemophilia a from other hemophilias

A

severe GIT and intracranial hemorrhage

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63
Q

: with GIT bleeding and intracranial haemorrhage

A

FVIII

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64
Q

: without GIT bleeding and intracranial haemorrhage

A

FIX

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65
Q

VIII:C belongs in the intrinsic pathway

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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66
Q

: to arrest bleeding

A

cryoprecipitate

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67
Q

cryoprecipitate Contains

A

VIII, vWF, fibrinogen, fibronectin

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68
Q

Effected for qualitative defect of VIII:C

A

cryoprecipitate

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69
Q

Effected for qunlitative defect of VIII:C

A

Prophylactic DDAVP

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70
Q

Prophylactic: to prevent after encounter

A

Prophylactic DDAVP

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71
Q

Administered after uncommon bleeding disorder

A

Prophylactic DDAVP

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72
Q

Analogous to VIII:C – increase and stimulate endothelium to release VIII:C complex o raise VIII:C by 3-6x fold

A

Prophylactic DDAVP

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73
Q

Prophylactic DDAVP

A

Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

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74
Q

What is the effect of DDAVP therapy?

A
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75
Q

What coagulation test will be prolonged? Hemophilia A/Classic Hemophilia (Factor VIII:C Deficiency)

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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76
Q

▪ sex-linked disorder characterized by a deficiency of Factor IX

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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77
Q

▪ second most common hemophilia

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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78
Q

▪ activation: XI, K, VII

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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79
Q

▪ role: to form intrinsic tenase in activation of FX

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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80
Q

▪ clinical findings:
- mild to severe bleeding disorder
- no GIT and intracranial involvement
- severe conditions may be indistinguishable from Hemophilia A

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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81
Q

▪ laboratory findings: prolonged APTT

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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82
Q

▪ correction: aged serum (any reagent plasma w/ FIX)

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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83
Q

▪ not corrected by: adsorbed plasma (no prothrombin group)

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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84
Q

▪ treatment/therapy:
• Factor IX commercial concentrate
• Fresh frozen plasma

A

Hemophilia B/Christmas disease (Factor IX Deficiency)

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85
Q

IX can be activated by

A

XI, K, VII

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86
Q

o isolated from plasma

A

Factor IX commercial concentrate

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87
Q

o in blood donation: separation of packed rbc from plasma from donor and recipient →pooled plasma→isolation of FIX

A

Factor IX commercial concentrate

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88
Q

Hemophilia B/Christmas disease (Factor IX Deficiency)
What coagulation test will be prolonged?

A
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89
Q

→ These are autosomal recessive disorders with no associated bleeding tendencies. Patients are more vulnerable to excessive clotting (THROMBOSIS)

A

Prekallikrein; HMWK; Factor XII deficiency

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90
Q

Prekallikrein; HMWK; Factor XII deficiency
What coagulation test will be prolonged?

A
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91
Q

Autosomal recessive

A

II
VII
X
V
XIII

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92
Q

-Autosomal recessive -Autosomal dominant

A

I

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93
Q

Prothrombin deficiency

A

II

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94
Q

F VII deficiency

A

VII

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95
Q

F X deficiency

A

X

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96
Q

-Afibrinogenemia
-Dysfibrinogenemia

A

I

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97
Q

Owren’s disease Labile factor deficiency Parahemophilia

A

V

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98
Q

F XIII deficiency

A

XIII

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99
Q

Liver disease Vit. K deficiency Oral anticoagulant therapy

A

II
VII
X

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100
Q

Severe liver disease DIC Fibrinolysis

A

I
V
XIII

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101
Q
  • inherited lack of fibrinogen
A

• Afibrinogenemia (recessive)

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102
Q
  • severe bleeding symptoms
A

• Afibrinogenemia (recessive)

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103
Q
  • inherited deficiency of fibrinogen
A

• Hypofibrinogenemia (dominant)

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104
Q
  • bleeding symptoms correlate with fibrinogen concentration
A

• Hypofibrinogenemia (dominant)

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105
Q

Soft tissue bleeding

A

Factor VII (stable factor) deficiency

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106
Q

Soft tissue bleeding and chronic bruising

A

Factor X (StuartPrower) deficiency

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107
Q

Mild to moderate bleeding symptoms

A

Factor V (Owren disease, labile factor) deficiency

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108
Q

Mild bleeding symptoms

A

Factor II (prothrom bin) deficiency

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109
Q

• spontaneous bleeding

A

Factor XIII (fibrinstabilizing factor) deficiency

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110
Q

• delayed wound healing

A

Factor XIII (fibrinstabilizing factor) deficiency

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111
Q

• unusual scar formation

A

Factor XIII (fibrinstabilizing factor) deficiency

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112
Q

• increased incidence of spontaneous abortion

A

Factor XIII (fibrinstabilizing factor) deficiency

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113
Q

spontaneous bleeding of mucosa, intestines, and intracranial sites

A

Factor I (fibrinogen) deficiency

114
Q

Prolonged PT

A

Factor VII (stable factor) deficiency

115
Q

Prolonged PT, aPTT, and Stypven Time/RVV T

A

Factor X (StuartPrower) deficiency

116
Q

Prolonged PT and aPTT

A

Factor V (Owren disease, labile factor) deficiency
Factor II (prothrom bin) deficiency

117
Q

• prolonged bleeding time (fibrinogen bridges do not form, platelet aggregation defect)

A

Factor I (fibrinogen) deficiency

118
Q

• decreased fibrinogen concentration

A

Factor I (fibrinogen) deficiency

119
Q

• prolonged PT, aPTT, and thrombin time

A

Factor I (fibrinogen) deficiency

120
Q

• 5.0 M urea test abnormal

A

Factor XIII (fibrinstabilizing factor) deficiency

121
Q

• PT and aPTT normal

A

Factor XIII (fibrinstabilizing factor) deficiency

122
Q

• Enzymatic and immunologic studies can be done

A

Factor XIII (fibrinstabilizing factor) deficiency

123
Q

only factor that prolongs PT when deficient; TF cannot be corrected in any way/uncorrecte d, VII – corrected Ca is involved in intrinsic tenase – APTT should also be prolonged

A

Factor VII (stable factor) deficiency

124
Q

aged serum

A

Factor VII (stable factor) deficiency
Factor X (StuartPrower) deficiency

125
Q

adsorbed plasma (PT becomes normal)

A

Factor V (Owren disease, labile factor) deficiency

126
Q

Not corrected by: adsorbed plasma (including other factor deficiencies in prothrombin group – II, IX, X)

A

Factor VII (stable factor) deficiency

127
Q

Not corrected by: adsorbed plasma

A

Factor X (StuartPrower) deficiency

128
Q

Treatment:
• fresh frozen plasma
• cryo-free or cryodepleted plasma

A

Factor V (Owren disease, labile factor) deficiency

129
Q

Note: very labile in storage (w/ VIII)

A

Factor V (Owren disease, labile factor) deficiency

130
Q

(to avoid disappearance of plasma ; raises FV levels and helps patient to demonstrate less symptoms)

A

fresh frozen plasma

131
Q

: left after some blood clotting proteins (cryoprecipitate) have been removed from fresh frozen plasma

A

cryo-free or cryodepleted plasma

132
Q

(provides purer and more adequate FV when defective;)

A

fresh frozen plasma

133
Q

contains functional coag factor

A

cryoprep

134
Q

Not in born; stimulated by external factors

A

Acquired Coagulation Disorders

135
Q

▪ majority of these substances are antibodies that targets proteins

A

Inhibitory Substances

136
Q

• stimulated by infusion of blood and the recipient rejects it, forming antibodies against it

A

Inhibitory Substances

137
Q

• release of tumor substances (recognized by the body as foreign thus it starts to attack and affects the coagulation factors)

A

Inhibitory Substances

138
Q

• common cause: autoimmune diseases (production of autoantibodies attacking one’s own cell; inhibits own coagulation factors)

A

Inhibitory Substances

139
Q

▪ also attacks one’s own cell thus inhibiting coagulation factors

A

Inhibitory Substances

140
Q

: directed to specific factors

A

▪ LA

141
Q

: directed to anti-PPL

A

▪ APA

142
Q

: directed to other coagulation factors

A

▪ Anticardiolipin antibodies

143
Q

directed against platelet phospholipid and phospholipid protein complexes (main target)

A

A. Non-specific inhibitor

144
Q

Laboratory: presence will prolong the PTT (due to lack of PPL) and dilute Russell viper venom test (problem w/ FX, which requires PPL in its activation)

A

A. Non-specific inhibitor

145
Q

▪ directed against specific coagulation factors:
• Factor VIII:C
• Factor IX
• Factor Xl

A

B. Specific factor Inhibitors/Circulating anticogulants

146
Q

Presence of FSP/FDP – seen in

A

fibrinolysis

147
Q

interfere with polymerization of fibrin strands when not cleared

A

FSP/FDP

148
Q

should only be present once cleaved by plasmin in an excess manner

A

FSP/FDP

149
Q

fragments are powerful anticoagulants and inhibitor of thrombin

A

FSP/FDP

150
Q

Tested by:
• Latex agglutination
• Measurement of fibrin monomers (quantitative)
• Platelet count
• Fibrinogen level assays
• PT and APTT (prolonged)

A

FSP/FDP

151
Q

functional decrease (since liver is normal and only vit. K is deficient) of II, VII, IX, X, Protein C, S and Z (Vit K dependent proteins)

A
152
Q

Causes:
• antibiotic intake
• decreased absorption
• antagonistic drugs

A

Vit K Deficiency

153
Q

also poses a problem w/ coauglation

A

Vit K Deficiency

154
Q

• necessary for the glutamic acid conversion into a gamma carboxyglutamic acid to enable the prothrombin group (II, VII, IX, X) to bind with calcium for coagulation process

A

Vitamin K

155
Q

o Vit K is produced by normal intestinal flora or normal flora in the GIT

A

antibiotic intake

156
Q

o Deficiency in normal flora = Vit K deficiency

A

antibiotic intake

157
Q

: Vit K is not processed in the liver; proteins are not passed in the liver and not processed well/broken down; problem in the liver; malabsorption of Vit K = Vit K deficiency

A

Obstructive jaundice

158
Q

: Vit K antagonists

A

warfarin, Coumadin

159
Q

▪ Breast-fed babies: more prone to vitamin K deficiency because breast milk is sterile, which allows no bacterial intestinal colonization to occur.

A

Vitamin K deficiency

160
Q

▪ Laboratory: Prolonged PT (VII, X, II) and prolonged aPTT (IX, X, II)

A

Vitamin K deficiency

161
Q

Vitamin K deficiency
What coagulation test will be prolonged?

A
162
Q

can result in decreased synthesis of proteins:
• coagulation factors
• fibrinolytic factors (Plasmin)
• regulatory/inhibitory proteins (anti-thrombin III, Protein C, Protein Z)

A

Liver/Hepatic Disease

163
Q

also causes impaired clearance of activated coagulation factors

A

Liver/Hepatic Disease

164
Q

most profound deficiency: FVII

A

Liver/Hepatic Disease

165
Q

Laboratory:
• Screening tests (Prolonged):
- PT
- aPTT
- Thrombin Clotting Time (TCT)

A

Liver/Hepatic Disease

166
Q

: to diagnose congenital or acquired fibrinogen deficiency

A

Thrombin Clotting Time (TCT)

167
Q

: major site of hemostatic protein synthesis

A

Liver

168
Q

activated coagulation factors uncleared in the circulation:
1. TPA
2. FSP

A

Liver/Hepatic Disease

169
Q

Normal: while coagulating, tissue plasminogen is prepared to bind w/ fibrin clot, making the clot dissolution on time and not delayed

A

TPA

170
Q

remains uncleared in the circulation after activation of plasminogen due to decreased synthesis of the regulatory protein TPAI = ↓ inhibitory proteins = TPA remains activated = causes excessive fibrinolysis

A

TPA

171
Q

FSP causes anticoagulation or cleaving of factor V, VIII, X, and I even without fibrin formation

A

Liver/Hepatic Disease

172
Q

(most labile with liver disease, Vit K deficiency, and intake of oral anticoagulant)

A

FVII

173
Q

Clinical: Soft tissue bleeding

A

Liver/Hepatic Disease

174
Q

Correction: aged serum

A

Liver/Hepatic Disease

175
Q

Not corrected by: adsorbed plasma (including other factor deficiencies in prothrombin group – II, IX, X)

A

Liver/Hepatic Disease

176
Q

Liver/Hepatic Disease
What coagulation test will be prolonged?

A
177
Q

replacement of more than 1.5 L blood volume in 24 hours

A

Massive Transfusion

178
Q

▪ results to DILUTION of coagulation factors and increased introduction of anticoagulants

A

Massive Transfusion

179
Q

▪ excess citrate decreases ionized calcium needed by the blood to clot

A

Massive Transfusion

180
Q

▪ most profound deficiency: V and VIII:C (labile factors upon storage)

A

Massive Transfusion

181
Q

: used to preserve blood, but chelates and binds to calcium; does not directly cause bleeding

A

Citrate

182
Q

in the bag used to transfuse the px could contaminate the blood and bind the Ca of the px

A

Citrate

183
Q

• Main: due to liberation of thromboplastic substance (containing TF) that activates coagulation

A

Disseminated Intravascular Coagulation (DIC)

184
Q

• activation of plasminogen through the contact phase, thus coagulation and fibrinolysis occur simultaneously and either may dominate

A

Disseminated Intravascular Coagulation (DIC)

185
Q

• consumption of coagulation factors and platelets (platelets are trapped in the clot produced by coag factors)

A

Disseminated Intravascular Coagulation (DIC)

186
Q

• RBC fragmentation (RBC - is affected due to clotting; does not flow well making it deformed and nonfuncitonal)

A

Disseminated Intravascular Coagulation (DIC)

187
Q

• Increased in FDP and D-dimer (D-dimer: indication of excess fibrinolysis)

A

Disseminated Intravascular Coagulation (DIC)

188
Q

Types of DIC

A
  1. Acute DIC
  2. Chronic DIC
189
Q

▪ appears within a few hours

A

Acute DIC

190
Q

▪ skin: purpura, gangrene, and bullae

A

Acute DIC

191
Q

▪ mortality: 60-80%

A

Acute DIC

192
Q

▪ less hemorrhage

A

Chronic DIC

193
Q

▪ thrombotic events (can lead to vascular occlusion)

A

Chronic DIC

194
Q

DIC Prolonged:

A

• TCT
• PT
• APTT

195
Q

DIC Decreased:

A

• Fibrinogen (decreased 4-24 hours after onset)
• Platelet
• AT-III

196
Q

DIC Increased:

A

• FSP
• Fibrin monomers

197
Q

DIC Positive:

A

• D-dimer test (due to presence of FSP)

198
Q

DIC
What coagulation test will be prolonged?

A
199
Q

▪ acid mucopolysaccharide that acts together with antithrombin III to inhibit thrombin

A

Heparin

200
Q

▪ used to treat thrombotic disorders caused by excess thrombin

A

Heparin

201
Q

▪ half-life: 3 hours

A

Heparin

202
Q

▪ antagonizes Vit K (factors are present but not functioning)

A

Coumarin Drugs: Warfarin

203
Q

▪ causes Protein induced by vitamin K absence (PIVKA)

A

Coumarin Drugs: Warfarin

204
Q

: powerful inhibitor of thrombin

A

Heparin together w/ antithrombin III

205
Q

Easy bruising

A

II, VIII, IX

206
Q

Delayed wound healing

A

I, XIII

207
Q

Hematomas

A

II, VIII, IX

208
Q

Umbilical cord bleeding

A

X, XIII

209
Q

Mucosal bleeding/Ecch ymosis

A

II, VIII, IX, XI

210
Q

Miscarriage

A

I, XIII

211
Q

Hemarthrosis

A

VIII, IX, X

212
Q

Thrombosis

A

Abnormal fibrinogens; LA; Inhibitor deficiencies

213
Q

Postsurgical bleeding

A

I, II, V, VII, VIII, IX, X, XI, XIII

214
Q

Asymptomati c

A

FXII, PK, HK

215
Q

Intracranial bleeding

A

VII, VIII, IX, XIII

216
Q

DISORDERS of Fibrinolysis

A

Primary fibrinolysis (fibrinogen lysis)

217
Q

▪ referred to as “Pathologic Fibrinolysis of PF”

A

Primary fibrinolysis (fibrinogen lysis)

218
Q

▪ pure form and unusual (lethal; life-threatening)

A

Primary fibrinolysis (fibrinogen lysis)

219
Q

▪ Cause: release of excessive amount of plasminogen activators (TPA) in circulation from damaged/malignant cells or ruptured vein resulting to the conversion of plasminogen to plasmin in the absence of fibrin formation (Ex. metastatic carcinoma)

A

Primary fibrinolysis (fibrinogen lysis)

220
Q

▪ Treatment: anti-fibrinolytic drugs/antiplasmin

A

Primary fibrinolysis (fibrinogen lysis)

221
Q

TPA should only activate plasminogen when there is a detected fibrin formation

A

Primary fibrinolysis (fibrinogen lysis)

222
Q

No clot formed, no activation of FI = activates independently due to excess ruptured/malignant cell

A

Primary fibrinolysis (fibrinogen lysis)

223
Q

Problem: excess TPA (activation of plasmin)

A

Primary fibrinolysis (fibrinogen lysis)

224
Q

▪ clot dissolution (excessive) results to increase FSP (spil products: D-dimer)/FDP (fragments: X and Y) that interfere with coagulation and platelet function Secondary fibrinolysis (fibrin lysis)

A

Secondary fibrinolysis (fibrin lysis)

225
Q

▪ occurs in DIC (simultaneous clotting and fibrinolysis)

A

Secondary fibrinolysis (fibrin lysis)

226
Q

Euglobulin Test markedly shortened

A

PRIMARY

227
Q

Euglobulin Test normal to slightly shortened

A

SECONDARY

228
Q

Platelet count > 100 x 109/L

A

PRIMARY

229
Q

Platelet count < 100 x 109/L

A

SECONDARY

230
Q

AT-III normal

A

PRIMARY

231
Q

AT-III decreased (due to FSP)

A

SECONDARY

232
Q

D-dimer Test negative

A

PRIMARY

233
Q

D-dimer Test positive

A

SECONDARY

234
Q

: compromises health and normal functioning of the body

A

PATHOLOGIC THROMBOSIS

235
Q

▪ can be acquired or inherited

A

PATHOLOGIC THROMBOSIS

236
Q

PATHOLOGIC THROMBOSIS risk factors include:

A

• Inherited/Congenital conditions
• Acquired conditions

237
Q

: related with congenital deficiencies in the regulatory proteins/inhibitors (antithrombin II, any antiplatelet reg proteins) and defective factors

A

• Inherited/Congenital conditions

238
Q

: related with lifestyle such as age, smoking and other diseases (immunocompromised)

A

• Acquired conditions

239
Q

▪ composed of platelets with small amounts of fibrin, red and white cells - “white clot”

A

Arterial thrombosis

240
Q

Arterial thrombosis causes:

A

• hypertension
• hyperviscosity
• qualitative platelet abnormalities
• atherosclerosis (accumulation of fats in blood vessels interfere with normal blood flow)

241
Q

▪ composed of large amounts of fibrin and red cells

A

Venous thrombosis

242
Q

▪ associated with slow blood flow, activation of coagulation, impairment of the fibrinolytic system, and deficiency of physiological inhibitors

A

Venous thrombosis

243
Q

recurrent venous thrombosis

A

Antithrombin (AT) Deficiency

244
Q

DIC, liver disease, nephrotic syndrome, oral contraceptives, and pregnancy

A

Antithrombin (AT) Deficiency

245
Q

Not associated with thrombosis

A

Heparin Cofactor II Deficiency

246
Q

thromboembolism

A

Protein C Deficiency

247
Q

Vitamin K deficiency, liver disease, malnutrition, DIC, and warfarin therapy

A

Protein C Deficiency

248
Q

vitamin K deficiency, liver disease, and DIC

A

Protein S Deficiency

249
Q

factor V Leiden is not inactivated → excessive clot formation

A

Activated Protein C Resistance (Factor V Leiden)

250
Q

increase in the concentration of plasma prothrombin

A

Prothrombin Mutations

251
Q

Other Inherited Thrombotic Disorders

A

• Elevated activity levels of factor VIlI are associated with venous thrombosis embolism.
• Factor XII deficiency
• Dysfibrinogenemia; Hyperhomocysteinemia
• Tissue factor pathway inhibitor (TFPI) deficiency

252
Q

– principal inhibitor of thrombin

A

• AT-III

253
Q

: recurrent deep venous thrombosis in anatomic sites (pulmonary embolism in lungs; thrombophlebitis in lower extremities)

A

• ↓ AT-III

254
Q

• normal value in vivo: 85-125%

A

AT-III:

255
Q

• moderate risk: 60-80%

A

AT-III:

256
Q

• significant risk: 50-60%

A

AT-III:

257
Q

• preferred sample for test: serum (reflects lower plasma AT-III level)

A

AT-III:

258
Q

• normal value in serum: 70- 125%

A

AT-III:

259
Q

prevent platelet activation and aggregation and are most effective in the treatment of the arterial diseases.

A

Antiplatelet Drugs

260
Q

irreversibly affects platelet function by inhibiting the cyclooxygenase (COX) enzyme and thereby the formation of thromboxane A2 (TXA2).

A

Aspirin (acetylsalicylic acid)

261
Q

: a potent activator of platelet

A

thromboxane A2 (TXA2)

262
Q

Other antiplatelet drugs include

A

dipyridamole, thienopyridines, ticlopidine and clopidogrel

263
Q

Acquired Thrombotic Disorders

A
264
Q

• Lupus anticoagulant does not inhibit in vivo coagulation but may cause prolonged in vitro tests

A
  1. Lupus Anticoagulant/ Antiphospholipid Syndrome
265
Q

• Development of antibodies against heparinplatelet factor 4 complex.

A
  1. HeparinInduced Thrombocytopenia
266
Q

• This immune complex causes platelet activation, platelet microparticles, thrombocytopenia, and hypercoagulable state.

A
  1. HeparinInduced Thrombocytopenia
267
Q

▪ inhibit thrombin and fibrin formation

A

Anticoagulant Drugs

268
Q

• the anticoagulant activity of heparin is enhanced by binding to AT.

A
  1. Intravenous anticoagulant: Heparin
269
Q

inactivates thrombin and factor Xa

A

• Heparin-AT complex

270
Q

is monitored by APTT & activated Clotting time

A

• Heparin dosage

271
Q

What is the treatment for heparin overdose?

A
272
Q

• inhibit vitamin K-dependent factors and other vitamin K-dependent proteins.

A
  1. Oral Anticoagulants: (Coumadin, Warfarin, Dicumarol)
273
Q

• alter hepatic synthesis resulting to inability of the liver to carboxylate the glutamyl residue of the factors leading to their functional deficiency.

A
  1. Oral Anticoagulants: (Coumadin, Warfarin, Dicumarol)
274
Q

These factors/proteins formed are referred to as PIVKA/des-y-carboxy proteins.

A
  1. Oral Anticoagulants: (Coumadin, Warfarin, Dicumarol)
275
Q

crosses the placenta and is present in human milk.

A

• Coumadin (warfarin)

276
Q

• Therapy is monitored by PT/international normalized ratio (INR).

A
  1. Oral Anticoagulants: (Coumadin, Warfarin, Dicumarol)
277
Q

What is the treatment for accidental warfarin poisoning?

A
278
Q
  • used to break down fibrin clots to restore vascular function and to prevent loss of tissues and organs
A

Thrombolytic Drugs

279
Q

also used in acute arterial thrombosis for immediate thrombolysis

A

Thrombolytic Drugs

280
Q

not fibrin specific (targets the whole clot in general)

A

Urokinase

Streptokinase

281
Q

used in the treatment of venous thromboembolism, MI, and thrombolysis of clotted catheters

A

Urokinase

Hematology 2 Lec (29 decks)

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