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Block 7 Week 4 > Coagulation-Fung > Flashcards

Coagulation-Fung Flashcards

1
Q

What are the three steps of normal hemostasis?

A

Vasoconstriction
platelet aggregation
fibrin formation

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2
Q

What are the three steps of primary hemostasis?

A
  1. adhesion
  2. degranulation
  3. aggregation
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3
Q

What is this:
with injury Von Willbrand Factor and collagen are exposed on the subendothelial basement membrane
Platelet receptor GPIb binds to Von willebrand factor

A

Adhesion

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4
Q

What is this:

  • platelets release their alpha and dense granules
  • synthesize and release TXA2
A

Degranulation

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5
Q

What is this:

-Platelet receptor GPIIB/IIIa and fibrinogen allows platelets to aggregate to one another

A

aggregation

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6
Q

Where are almost all factors except for Von willbrand factor produced?

A

liver

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7
Q

Where is von willebrand factor produced?

A

endothelial cells

megakaryocytes

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8
Q

What is another name for factor I?

A

fibrinogen

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9
Q

What is another name for factor II?

A

Prothrombin

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10
Q

What is another name for factor VIII?

A

antihemophilic factor

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11
Q

What is another name for factor IX?

A

christmas factor

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12
Q

What is another name for factor XII?

A

Hagemann factor

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13
Q

What is another for factor XIII?

A

Fibrin stabilizing factor

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14
Q

What is the common pathway for the intrinsic and extrinsic pathways of coagulation?

A

factor X-> Xa

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15
Q

When wil the intrinsic coag cascade happen?

A

with contact phase

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16
Q

When will the extrinisc coag cascade happen?

A

tissue injury and tissue factor

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17
Q

What is the intrinisc pathway?

A

XII->XIIa-> which converts IX to IXa and VIII is converted to VIIIa by IIa. VIIIa and IXa will make X which will go to Xa which will convert prothrombin (II) to thrombin (IIa) which will convert fibrinogen to fibrin. Factor XIII will then turn into factor XIIIa which wil convert fibrin into cross linked fibrin

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18
Q

What is the extrinsic pathway?

A

VII is converted to VIIa which converts X to Xa* which will combine with Va* (made from IIa converting V to Va)
Va and Xa* convert prothrombin (ii) into thrombing (iia) which converts fibrinogen to fibrin which when combined with XIIIa will create cross linked fibrin

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19
Q

What makes up the prothrombinase complex?

A

Va and Xa

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20
Q

What makes up the tenase complex?

A

VIIIa, Xa, Ca

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21
Q

What is this:

the balance between clotting and thrombosis

A

hemostasis

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22
Q

Hemostasis is a precisely orchestrate process involving (blank), (blank), and (Blank)

A

pateletes
clotting factors
endothelium

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23
Q

Where does hemostasisi occur?

A

at site of vascular injury and culminates in the formation of a blood clot

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24
Q

What are 2 disorders of hemostasis?

A

hemorrhagic disorders

thrombotic disorders

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25
Q

What is this:

characterized by excessive bleeding

A

Hemorrhagic disorders

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26
Q

What is this:

Characterized by clot formation

A

thrombotic disordes

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27
Q

Why do we get vasoconstriction in hemostasis and how?

A

-reduces blood flow to area, -mediated by reflex neurogenic mechanisms and augmented by local secretion of endothelin
(transient effect)

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28
Q

What is primary hemostasis?

A
  • platelets adhere to endothelium and are activated

- this process leads to agregation and the beginning of a clot

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29
Q

What is secondary hemostasis?

A
  • tissue factor is exposed at the site of vascular injury

- sets in motion a cascade of reactions that leads to thrombin fomration

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30
Q

(blank) release causes vasoconstriction

A

endothelin

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31
Q

At the site of injury you will get reflex (Blank)

A

vasoconstriction

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32
Q

THe blood vessel consists of three layers what are they?

A

Intima – contains endothelium
Media – layer of smooth muscle
Adventitia – rich in connective tissue

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33
Q

Vascular endothelium is a dynamic organ that actively regulates hemostasis by ….?

A
  • inhibiting platelets
  • suppressing coag
  • promoting fibrinolysis
  • mod vascular tone and permeability
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34
Q

What are the anti-thrombotics for blood vesses?

A 
heparin
protein C and protein S
tissue plasminogen activator
prostacyclin
nitric oxide
thrombomodulin
protein C receptor
Tissue factor pathway inhibitor (TFPI)
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35
Q

What are the prothrombics for blood vessels?

A 
Factor V
tissue factor
plasminogen activator inhibitor-1 (PAI-1)
thromboxane
platelet activating factor
vWF
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36
Q

Upon vascular injury, what happens?

A
  • tissue factor is released
  • extracellular matrix (collagen) is exposed
  • media and adventitia mediate vasoconstriction
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37
Q

Blood vessel exposure to (blank) and (blank) sets the stage for platelets adherence and activation

A

collagen and vWF

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38
Q

Give me the 5 steps of primary hemostasis?

A

1) platelet adhesion
2) shape change
3) granule release (ADP, TXA2)
4) recruitment
5) aggregation (hemostatic plug)

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39
Q

(blank) are derived from megakaryocyte maturation

A

platelets

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40
Q

What is this:

anucleate structure consisting of a cytoskeleton and cytoplasmic granules

A

Platelets

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41
Q

What are the components of the cytoplasm?

A 
GP Ib/V/IX
GP IIb/IIIa
GP Ic/IIa
CD62P (p selectin)
Thrombin receptor
ADP receptor
GP Ia/IIa
Red cell antigens
Class I MHC antigen
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42
Q

What does G Ib/V/IX bind?

A

vWF

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43
Q

What does GP IIb/IIIa bind?

A

fibrinogen

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44
Q

What does GP Ic/IIa bind?

A

fibrinonectin

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45
Q

What does CD62p (p selectin) bind?

A

adhesion molecule

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46
Q

What does the thrombin receptor bind?

A

initiates platelet activation when bound by thrombin

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47
Q

What does the ADP receptor bind?

A

initiates platelet activation when bound by ADP

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48
Q

What does GP Ia/IIa bind?

A

binds collagen

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49
Q

What do red cell antigens bind?

A

ABO, P, I, i, le (no Rh)

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50
Q

What does Class I mHC antigen bind?

A

HLA-A, HLA-B, HLA-C

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51
Q

What are the alpha granules of platelets (about 75-80 per cell)?

A 
vWF
Fibrinogen
Factor V
VEGF, EGF, PDGF
angiostatin, thrombospondin, endostatin
PF-4, IL-8, CCL-5
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52
Q

What are the dense granules of platelets (less than 12 per cell)?

A

ATP
ADP
Calcium
Serotonin

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53
Q

Platelets adhere to the exposed (blank) and (blank) at the site of injury.

A

collagen

vWF

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54
Q

Platelet adhesion is mediated by platelet (blank) binding to vWF

A

GP Ib/V/IX

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55
Q

Platelet activation leads to…?

A

shape change
release of alpha and dense granules (ADP)
initiation of thromboane A2 pathway
GP IIb/IIIa to change to its active form

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56
Q

What are the four steps to secondary hemostasis?

A

1) tissue factor
2) phospholipid expression
3) thrombin activation
4) fibrin polymerization

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57
Q

Okay so say you have a tissue injury and you have collagne and VWF exposed, then what?

A

Gp1a and GP1b binds and then the platelet releases ADP, TXA2, 5-HT which stimulates the platelet to become activated and degranulate

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58
Q

What does antithrombin regulate?

A 
Thrombin
FIXa
FXa
FXIa
FXIIa
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59
Q

What does activated protein C (APC) regulate?

A

FVa

FVIIIa

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60
Q

What is the tissue factor pathways inhibitor/extrinisc pathway inhibitor?

A

tissue factor-FVIIa-FXa

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61
Q

What does plasmin do?

A

degrades fibrin (PA converts plasminogen to plasmin)

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62
Q

Evalutation of hemostasis can be divided into what 2 categories?

A

platelets

coagulation

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63
Q

What are the studies you can do on platelets to evaluate hemostasis?

A
  • platelet cout
  • bleeding time
  • platelet aggregometry (function test)
  • platelet flow cytometry
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64
Q

What are the studies you can don on coagulation to evaluate hemostasis?

A

aPTT
PT
Thrombin Time
others

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65
Q

What is bleeding time used for?

A

used to screen qualitative platelet disorders or von willebrand disease
(dont used in patient w/out a hitory of excessive bleeding0

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66
Q

Is bleeding time a useful test to assess risk of bleeding during surgery?

A

no

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67
Q

What is the normal range of bleeding time?

A

1.5-9.5 minutes

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68
Q

Where will you see prolongations in bleeding time?

A
  • von willebrand disease
  • inherited platelet disorders
  • uremia
  • aspirin ingestion
  • low platelet counts (<100,000)
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69
Q

What is platelet aggregometry?

A

in vitro evaluation of platelet aggregation

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70
Q

Where do you perform platelet aggregometry?

A

performed on platelet-rich plasma that is exposed to various agonists such as ADP, epinephrine, arachidonate, collagen, ristocetin

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71
Q

What is platelet flow cytometry used for?

A
  • to diagnose deficiencies of platelet surface glycoproteins

- disorders of platelet activation

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72
Q

How does platelet flow cytometry work?

A

detects cell surface proteins w/ fluorescently labeled antibodies

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73
Q

What how do you determine platelet count and what is normal?

A

w/ an automated counter

Referance range: 140-400 X 10^9 cells/mL

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74
Q

What is prothrobin time (PT) used for?

A

used to evaluate the extrinsic and common pathways

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75
Q

How does prothrombin time test work?

A

tissue factor and thromboplastin added to plasma w/ excess calcium and the time to clot is measured

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76
Q

What is the reference range of prothrombin time?

A

11-13.5 seconds

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77
Q

What can cause prolongation of coagulation?

A 
deficiencies and inhibitors of:
Factor VII
Factor X
Factor V
Factor II
Fibrinogen
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78
Q

What do you use to monitor warfarin (coumadin) therapy?

A

prothrombin time

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79
Q

What is the INR (international normalized ratio) for?

A

used to reduce interlab variation that results from differing thromboplastin sensitivities to coumadin

80
Q

What is used as a valid standardizing calculation in coumandized patients with PT in the low range

A

INR (international normalized ratio)

81
Q

What is activatd partial thromboplastin time (aPTT)?

A

used to evaluate intrinsic and common pathways

82
Q

How does the activated partial thromboplastin time test work?

A

Phosopholipid is added to plasma with excess calcium and the time to clot is measured.

83
Q

What is the reference range for activatd partial thromboplastin time (aPTT)?

A

27-35 seconds

84
Q

Why would you have prolongation in activated partial thromboplastin time (aPTT)?

A 
Deficiencies and inhitors of :
Factor XII
Factor IX
Factor X
Factor II
Factor XI
Factor VIII
Factor V
Fibrinogen
85
Q

What is activated partial thromboplastin time (aPTT) used to monitor?

A

heparin, hirudi, or argatroban therapy

86
Q

What is thrombin time (TT) used for?

A

to test for the presence of functional fibrinogen

87
Q

How do you do the thrombin time (TT) test?

A

thrombin is added to patient’s plasma and the time to clot is measured

88
Q

What is the reference range of thrombin time (TT)?

A

15-19 seconds

89
Q

What is prolongation in thrombin time (TT) caused by?

A

heparin, hirudin, argatroban and dysfibrinogenemia

90
Q

What are mixing studies for?

A

Used to deterine cause of prolonged clotting times (PT, PTT,TT) AND
to screen for the presence of inhibitors

91
Q

What is the process behind mixing studies?

A
  • exclude the presence of heparain through heparin neutralization
  • mix patient’s plasma w/ normal test plasma, incubate and repeat test
92
Q

If you do a mix study and there is at least 50% activity (correction of value) then what does this mean?

A

coagulation prolongation is due to a factor deficiency

93
Q

If you do a mix study and there is less than 50% activity (correction of value), prolongation is due to presence of a (blank)

94
Q

What does the presence of D-dimers show us?

A

formd by plasmin mediated degredation of fibrin, presence indicates that fibrin has been formed and degraded (clotting)

95
Q

What is the von Willebrand factor assay (ristocetin cofactor assay)?

A

Version of platelet aggregation where patient plasma is added to formalin-fixed normal platelet in the presence of ristocetin. Normal patients will have platelet agglutination but vWF deficient patients will have decreased agglutination

96
Q

What is the Anti-Xa assay for?

A

used to monitor either unfractionated or low molecular weight heparin

97
Q

What are factor assays for?

When do yo do it?

A

to assess individual factor activity

Done after an intial mixing study shows a factor deficiency!!

98
Q 
What are these:
Factor VIII inhibitor assay
Fibrinogen activity
Protein C activity
Protein S activity
A

Other assays

99
Q

Clinically most bleeding disorders present as either (blank) or (blank) bleeding

A

platelet-type bleeding

coagulation-type bleeding

100
Q

If you see petechia what type of bleeding disorder should you be thinking of?

A

Platelet-type bleeding disorder

101
Q

If you see hemarthroses, what type of bleeding disorder should you be thinking of?

A

coagulation-type bleeding disorder

102
Q

If you see deep hematomas, what type of bleeding disorder should you be thinking?

A

coagulation-type bleeding disorder

103
Q

If you see delayed bleeding, what type of bleeding disorder should you be thinking?

A

coagulation-type bleeding disorder

104
Q

If you see mucosal bleeding what type of bleeding disorder should you be thinking?

A

platelet-type bleeding disorder

105
Q

If you have a female with a bleeding disorder what should you be thinking?

A

platelet-type bleeding disorder

106
Q

If you have a male with a bleeding disorder what should you be thinking?

A

coagulation-type bleeding disorder

107
Q

What are the diseases associated with platelet defects?

A 
von Willebrand disease
Bernard Soulier syndrome
Glanzmann thrombastena
Platelet storage pool disorders
Drugs
Immune thrombocytopenic purpura (ITP)
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic uremic syndrome (HUS)
Heparin induced thrombocytopenia (HIT)
108
Q

What are the diseases associated with coagulation defects?

A 
von Willebrand disease
Hemophilia A
Hemophilia B
Liver disease
Vit K deficiency
Disseminatd intravascular coagulation (DIC)
109
Q

What is the most common inherited bleeding disorder (1% of population)?

A

von willebrand disease

110
Q

What is the defect in von Willebrand disease?

A

defect of platelet adhesion

111
Q

What kind of symptoms do you get in von Willebrand disease?

A

combined platelet and coagulation defect so patient presents with both types

112
Q

What are the four main types of von willebrand disease?

A

Type I
Type II
Type III (severe disorder with no vWF)
Pseudo (platelet type) -abnormal platelet GPIb leading increased binding of vWF

113
Q

Where do you synthesize von Willebrand factor?

A

in endothelial cells and megakaryocytes

114
Q

Where is von Willebrand factor stored?

A

in Weibel-Palade bodies of endothelial cells and agranules of megakaryocytes

115
Q

What does von willebrand do?

A

mediates platelet adhesion by binding GPIb

116
Q

How does von willebrand factor circulate?

A

as variably sized multimers

117
Q

Von willebrand factor is also called (Blank)

A

FVIII-related antigen (complexes with FVIII in circulation)

118
Q

vWF activity is referred as (blank) cofactor activity

A

ristocetin

119
Q

What is the most common type of von willebrand disease?

120
Q

Von Willebrand disease is a (blank) disorder showing reduced amounts of vWF

A

quantitative

121
Q

What will lab eval of von willebrand disease show?

A

prolonged PTT and BT
Decreased FVIII
Decreased vWF and activity

122
Q

What kind of defect does type II von willebrand disease have?

A

qualitative (functional) defect of vWF

123
Q

What are the four subtypes of type II von willebrand disease?

A

IIa
IIb
IIM- rare defect that prevents vWF binding to GPIb
IIN-rare defect w/ reduced vWF binding to FVIII

124
Q

What is this:
moderately severe disorder (10-15%)
absence of high molecular weight multimers

A

Type IIa von willebrand disease

125
Q

What Is the lab eval for type IIa von willebrand disease?

A

PTT slightly prolonged

Ristocetin cofactor activity decreased

126
Q

What is this:
results from spontaneous binding of vWF to platelets
dereased high molecular weight multimers
do not give DDAVP (profound thrombocytopenia and bleeding)
Lab eval: enhanced ristocetin cofactor activity

A

Type IIb von willebrand disease

127
Q

What is this:
defect of platelet adhesion
due to decreased platelet GPIb/V/IX
patient clinically manifest with thrombocytopenia and giant platelets

A

Bernard Soulier Syndrome

128
Q

What are the lab evals of Bernard Soulier syndrome?

A

PFT: no aggregation w/ ristocetin

Peripheral smear will show large platelets (unlike vWD)

129
Q

What is this:
Autosomal recessive disorder due to deficient GP IIb/IIIa (fibrinogen receptor)
Platelets lack the PLa1 antigen
Lab eval shows failure to aggregate with all agonists but ristocetin

A

Glanzmann thrombasthenia

130
Q

What do patients lack in glanzmann thrombasthenia?

A

platelets lack of PLa1 antigen

131
Q

In the platelet storage pool defects, what are dense granule diseases and what are they?

A

no second wave of aggregation

  • Hermansky-Pudlak
  • Chediak-Higashi
  • Wiskott-Aldrich
132
Q

In the patelet storage pool defects, what are alpha-granule diseases?

A

aggregation is blunted with all agents except ADP

-gray platelet syndrome

133
Q

What are drugs that can cause excessive bleeding?

A

Aspirin and NSAIDS

134
Q

What does Aspirin and NSAIDS do?

A

inhibit COX-1 (enzyme involved in TXA2 production)

135
Q

(blank) exerts autocrine platelet stimulation leading to dense granule release and the secondary wave of aggregation

136
Q

The aspirin effect is (reversible/irreversible)

A

irreversible

137
Q

The NSAID effect is (reversible/irreversible)

A

reversible

138
Q

What drugs are these:
cause inhibition of ADP-mediated platelet activation
-will see blunted platelet aggregation on PFT in response to ADP

A

Ticlopidine and Clopidogrel

139
Q

What drugs are these:
inhibitors of GP IIb-IIIa (fibrinogen receptor)
-will see impaired aggregation to all agonists except ristocetin on PFT

A

Abciximab, Eptifibatide, TIrofiban

140
Q

What are some disorders characterized by thrombocytopenia?

A

Immune thrombocytopenic purpura (ITP)
thrombotic thrombocytopenic purpura (TTP)
Hemolytic uremic syndrome (HUS)
Heparin induced thrombocytpenia (HIT)

141
Q

What is immune thrombocytopenic purpura (ITP)?

A

Diagnosis of exclusion: healthy patient with isolated thrombocytopenia with no other obvious cause

142
Q

What is the antigenic target for ITP?

A

GPIIb
GPIIIa
GP Ib
GP V

143
Q

What is this:
syndrome resulting from widespread formation of microvascular platelet thrombi affecting CNS, kidneys, GI tract other organs

A

Thrombotic thrombocytopenic purpura (TTP)

144
Q

What are the symptoms of thrombotic thrombocytopenic purpura (TTP)?

A

Presents with a pentad of symptoms:

  • thrombocytopenia
  • microangiopathic hemolytic anemia
  • neurologic abnormalities
  • renal abnormalities
  • fever
145
Q

What is this:
usually idiopathic and an isolated event
-antibodies to the enzyme cause a dysfunction

A

Thrombotic thrombocytopenic purpura (TTP)

146
Q

What is the pathogenesis behind thrombotic throombocytopenic purpura (TTP)?

A
  • acute deficiency of vWF-cleaving metalloprotease (ADAMTS13)
  • causes an accumulation of ultra-large vWF multimers which bind platelets and lead to both thrombi in the microvasculature and thrombocytopenia
147
Q

(Blank) is a disorder that usually occurs when an infection in the digestive system produces toxic substances that destroy red blood cells, causing kidney injury.

A

Hemolytic-uremic syndrome (HUS)

148
Q

What is thrombotic microangriopathy characterized by?

A

Thrombocytopenia
Acute renal failure
Microangiopathic hemolytic anemia

149
Q

Hemolytic uremic syndrome is a thrombotic (blank)

A

microangiopathy

150
Q

What is hemolytic uremic syndrome most commonly associated with?

A

bloody-diarrhea caused by shiga-toxin producing bacteria

151
Q

What is this:
occurs in 1-3% of patients treated with unfractionated heparin for greater than 5 days.
-

A

Heparin induced thrombocytopenia (HIT)

152
Q

What causes heparin induced thrombocytopenia (HIT)?

A

an immune mediated disorder caused by IgG antibodies against the complex of heparin and PF4

153
Q

In heparin induced thrombocytopenia (HIT), which is more common bleeding or thrombosis?

A

thrombosis

bleeding is rare

154
Q

Why do you get thrombosis in heparin induced thrombocytopenia (HIT)?

A

due to platelet aggregation and activation of coagulation

155
Q

What are the symptoms of heparin induced thrombocytopenia?

A

thrombocytopenia
thrombosis
allergic reactions

156
Q

What is hemophilia A due to?

A

a congenital X-linked recessive deficiency of Factor VIII

157
Q

What does hemophilia A result in?

A

decreased or absent circulation FVIII resulting in lifelong bleeding

158
Q

In hemophilia A, patients clinically present with what?

A

delayed bleeding

joint and muscle bleeding

159
Q

What is the lab evaluation for hemophilia A?

A

increased PTT
normal platelet count, PT and TT
decreased plasma FVIII assay

160
Q

What is hemophilia B due to?

A

a congenital X-linked recessive deficiency of factor IX

161
Q

What does hemophilia B result in?

A

decreased or absent FIX leading to lifelong bleeding

162
Q

What is another name for hemophilia B?

A

christmas disease

163
Q

Is hemophilia B clinically distinguishable from hemophilia A?

A

no (use see delayed bleeding and joint and muscle bleeding)

164
Q

What is the lab eval for excessive bleeding?

A

prolonged PTT
Normal PT, TT
decreased FIX assay

165
Q

WHat is this:

decreased synthesis of most clotting factors, including fibrinogen

A

liver disease

166
Q

In liver disease you get chronic (blank) due to impaired clearance of D-dimer

167
Q

What does the lab eval look like?

A

Prolonged PT

prolonged TT

168
Q

(blank) is involve in y-carboxylation of the G1a domains of clotting factors.

169
Q

Vit K deficiency leads to impaired production of vit K dependent factors. What are these factors?

A 
Factor II
Factor VII
Factor IX
Factor X
Protein C
Protein S
170
Q

What causes vit K deficiency?

A
  • hemorrhagic disease of the newborn
  • antibiotics
  • malabsorption/malnutrion
  • warfarin therapy
171
Q

What is this:

acquired syndrome characterized by the intravascular activation of coagulation

A

Disseminated intravascular coagulation (DIC)

172
Q

What are the causes of DIC?

A 
Endotoxin causing sepsis (most common)
Trauma
Burns
Obstetrical complications
Vascular malformations
Animal venom
Mucin secreting adenocarcinoma
173
Q

Explain how DIC happens

A
  • Excess or prolonged exposure to tissue factor
  • generalized activation of coag system and thrombin generation
  • fibrin formation and fibrinolysis causes microthrombi and consumption of clotting factors and platelets
  • no activation of tissue factor pathway inhibitor (due to ongoing exposure to tissue factor)
174
Q 
What are these:
Hemorrhage
Renal dysfunction
Hepatic dysfunction
Respiratory dysfunction
Shock 
CNS dysfunction
Petechiae
Purpura
Skin necrosis 
Signs of thrombosis
A

Clinical manifestations of DIC

175
Q

If you suspect DIC, how should your lab evals go?

A

order coag tests: (platelet count, prothrombin time, fibrinogen, fibrin monomors or degredation products)
Score coag tests-> score greater than 5 compatible with overt DIC repeat scoring daily, less than 5 not affirmative for non-overt DIC repeat next 1-2 days

176
Q

Diffrential diagnosis of thrombophilia depends on the type of (blank)

A

thrombosis (arterial or venous)

177
Q

What are examples of excessive thrombosis?

A
  • activated protein C resistance (factor V leiden)
  • anti-thrombin deficiency
  • anti-phospholipid syndrome
  • prothrombin variant
  • protein C/protien S deficiency
178
Q

What is this:

inherited autosomal dominant condition responsible for 50% of the cases of hereditary thrombophilia

A

Activated protein C resistance (APCR)

179
Q

Activated protein C resistance (APCR) is due to a point mutation in the (blank) gene

180
Q

Activated protein C resistance is due to a point mutation in the FV gene that makes what happen?

A

FV leiden resistant to proteolytic cleavage by APC

181
Q

Heterozygotes for a FV gene mutation have a (blank) increased risk of thrombosis

182
Q

What is this:

autosomal dominant disorder characterized by recurrent venous thrombosis

A

Anti-thrombin deficiency

183
Q

What happens if you have an anti-thrombin deficiency?

A

you have no inactivation of factors II, IXA, Xa, XIa, XIIA (so you keep clotting)

184
Q

People with anti-thombin deficiency that are heterozygotes have (blank) increased risk of thrombosis. (Blank) is considered incompatible with life.

A

5-10

incompatible

185
Q

What is this:
an autoimmune thrombophilic condition in which patients have circulating antibodies against plasma proteins that bind to phospholipids.

A

Anti-phospholipid syndrome

186
Q

In anti-phospholipid syndrome. patients have what sort of problems?

A

recurrent arterial and venous thrombosis

  • pregnancy loss
  • immune cytopenias
187
Q

In antiphospholipid syndrome, antiphospholipid antibodies are directed toward (bank) (blank). How can you identify the antibodies?

A

B2 glycoprotein
prothrombin

By which coagulation assays they prolong

  • lupus anticoagulant
  • anticardiolipin
188
Q

What is primary APL and where do you find it?

A

Antiphospholipid syndrome in healthy individuals

189
Q

What is secondary APL associated with?

A

systemic lupus
malignancy
HIV
collagen vascular disease

190
Q

What drugs are associated with secondary APL?

A

Phenyotoin, quinidine, hydralazine, procainamide, phenothiazines, IFN, cocaine

191
Q

What is this:

autosomal dominant condition due to a point mutation in the prothrombin gene.

A

Prothrombin Variant (G20210A)

192
Q

What does the prothrombin variant mutation cause?

A

enhances prothrombin gene transcription leading to elevated levels of prothrombin

193
Q

What is the second most common cause of inherited thrombophilia?

A

Prothrombin variant

194
Q

What is this:

inherited autosomal dominant form with heterozygotes with 5-7 fold increased risk of thrombosis

A

Protein C/S deficiency

195
Q

The acquired form of Protein C/S deficiency can result from what?

A

Coumadin therapy
Liver disease
pregnancy

196
Q

Protein C/ S deficiency can either be due to (blank) or (blank) defects

A

qualitative or quantitative

Block 7 Week 4 (7 decks)

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