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Flashcards in Hemostasis and Related Disorders Deck (165)
1

What molecule mediates the transient vasoconstriction of primary hemostasis? What cell type releases this molecule?

endothelin

endothelial cells

2

What clotting factor binds subendothelial collagen?

vWF

3

Platelets bind vWF via what receptor?

GpIb

4

What two molecules are contained within Weibel Palade bodies?

vWF and P-selectin

5

What is contained in dense granules? What cell type?

ADP and calcium

platelets

6

GpIIb/IIIa is upregulated on the surface of platelets by what signal?

ADP binding ADP receptor

7

What molecule binds to GpIIb/IIIa?

fibrinogen

8

What are petechiae indicative of?

thrombocytopenia

9

What is a normal platelet count? Below what level can lead to petechiae?

150 - 400

10

What isotype of immunoglobulin is produced during Immune Thrombocytopenia? Against what?

IgG

GpIIb/IIIa

11

What cell type produces the Ab during Immune Thrombocytopenia? What organ is this cell located? What organ consumes these RBCs?

Plasma cells

spleen

spleen

12

Acute Immune Thrombocytopenia arises in what patient population? After what two happenings?

children

viral infection or vaccination

13

Chronic Immune Thrombocytopenia arises in what patient population?

women of childbearing age

14

Are any coagulation factors affected during Immune Thrombocytopenia? Why?

no . only destruction of GpIIb/IIIa

15

What is the initial treatment of Immune Thrombocytopenia?

corticosteroids = initial Tx

16

What two reasons is a splenectomy used as treatment for Immune Thrombocytopenia?

removes source of auto-Ab (splenic plasma)

removes site of platelet destruction (splenic macrophages)

17

What is microangiopathic hemolytic anemia?

pathological formation of platelet microthrombi in small vessels

18

What cellular happening is seen during MAHA?

schistocytes

19

What are the two classic disorders that will produce Microangiopathic Hemolytic Anemia?

TTP and HUS

20

Lack of what enzyme causes TTP? What is the normal function of this enzyme?

ADAMTS13

cleave vWF

21

What patient population most often develops auto-ADMATS13 Ab's?

adult female

22

What specifc bacterial strain produces HUS? Where is this strain found?

E. coli O157:H7

undercooked beef

23

Does HUS cause renal or CNS defects?

renal

24

Does TTP cause renal or CNS defects?

CNS

25

Are coagulation factors effected during HUS or TTP? What does this mean?

no

PT/PTT = normal

26

What cellular happening is observed during TTP/HUS?

schistocytes

27

What would be observed on a bone marrow biopsy of during TTP/HUS?

schistocytes

28

What are the two treatments of TTP and HUS?

plasmapheresis and corticosteroids

29

What is defective/absent during Bernard-Soulier syndrome? What is impaired?

GpIb

adhesion = impaired

30

What two cellular happenings are seen during during Bernard-Soulier syndrome?

mild thrombocytopenia and enlarged platelets

31

What is defective during Glanzmann thrombasthenia? What is impaired?

GpIIb/IIIa

aggregation = impaired

32

What activates factor VII?

tissue thromboplastin

33

What activates factor XII?

subendothelial collagen

34

Defects in primary homeostasis manifest as what? Where?

skin and mucosal bleeding

nose bleeds (Epistaxis)

35

Defects in secondary homeostasis manifest as what two conditions?

deep tissue bleeding

rebleeding after surgical procedures

36

PT measures which arm of the coagulation cascade?

extrinsic

37

Which clotting factors does PT measure?

I (fibrinogen), two, five, seven and ten

38

PTT measures which arm of the coagulation cascade?

intrinsic

39

What is the mode of inheritance of hemophilia A?

X-linked recessive

40

What are of the coagulation cascade would be effected during hemophilia A?

PTT

41

What coagulation factor is absent during Hemophilia B?

nine

42

What coagulation factor is most likely to develop an autoantibody against it? How would this be diagnosed?

eight

PTT does not correct upon mixing with normal serum

43

Will PTT be corrected if a patient with Hemophilia A is given normal serum?

yes

44

Will PTT be corrected if a patient with Coagulation Factor Inhibitor is given normal serum?

no

45

What two sites will bleed during vWF Disease?

skin and mucosa

46

What branch of the coagulation cascade will vWF Disease effect? Why?

PTT

vWF binds and protects factor eight

47

What drug is used to Dx vWF Disease? What is the MOA of this drug?

Ristocetin

causes vWF to bind GpIb

48

What drug is used to Tx vWF Disease? What is the MOA of this drug?

Desmopressin (DDAVP)

desmopressin causes endothelial cells to release vWF from Weibel-Palade bodies

49

What factors are regenerated by Vitamin K?

two, seven, nine, ten, C and S

50

What branch of the coagulation cascade is used to follow liver failure?

PT

51

What protein do platelets bind during HIT?

PF4

52

What isotype of Ab is involved during HIT?

PF4

53

What pro-coagulant is contained in amniotic fluid?

tissue thromboplastin

54

What two bacteria are the most common causes of septic DIC?

E. coli

N. meningitidis

55

Why does sepsis cause DIC? Why?

endotoxin

influences endothelial cells to make tissue factor

56

What coagulation factor is tissue factor?

III

57

What type of neoplasia can result in DIC? What specific product causes this?

adenocarcinoma

mucin

58

What type of leukemia can cause DIC? Why?

acute promyelocytic leukemia

primary granules

59

What type of bite can cause DIC?

rattlesnake

60

What is the serologic marker for DIC? What is D-Dimer derived from?

D_DIMER

fibrin split products

61

What surgery can cause a disorder of fibrinolysis? Why?

radical prostatectomy

release of urokinase

62

What if the function of urokinase?

activate plasmin

63

Why can liver failure result with a defect in fibrinolysis?

decrease production of α2 anti-plasmin during liver failure

64

Why do errors in fibrinolysis produce an increase in PT and PTT?

plasmin degrades coagulation factors

65

Is the platelet count normal during errors of fibrinolysis?

yes

66

What is the Tx of disorders of fibrinolysis? What is the MOA of this drug?

aminocaproic acid

blocks activation of plasminogen

67

What is the histological pathological finding of thombosis? What are the three components?

Lines of Zahn

platelets and fibrin and RBCs

68

What is Virchows triad?

stasis, endothelial damage, hypercoaguable state

69

What two anti-coagulant molecules are produced by healthy endothelial cells?

PGI2 and NO

70

What fibrinolytic molecule is secreted by a healthy endothelium?

tPA

71

What are the three functions of plasmin?

1. cleave fibrin and fibrinogen
2. destroy coagulation factors
3. block platelet aggregation

72

Does thrombomodulin activate Protein C or does Protein C activate thromobomodulin?

thrombomodulin activates Protein C

73

What coagulation factors are destroyed by Actiated Protein C?

five and eight

74

Deficiency of what vitamin can lead to endothelial damage?

B12

75

Deficiency of what enzyme can result in homocystinuria?

cystathione beta-synthase

76

What are the four defects of CBS deficiency?

vessel thrombosis

dislocated lens

mental retardation

long slender fingers

77

Deficiency of what two proteins predispose to warfarin induced necrosis? Why?

Protein C or Protein S

C and S have shorter half-lifes than two, seven, nine or ten

78

Why does warfarin induced skin necrosis happen?

microthrombi form in vessels

79

Why does Factor V Leiden produce hypercoaguable states?

Factor V lacks cleavage site for APC

80

How is antithrombin deficiency diagnosed?

heparin does not produce increase in PTT

81

What type of drug is associated with an increase in coagulation factor synthesis?

oral contraceptives

82

What is the characteristic histological feature of emboli?

cholesterol clefts

83

Fracture of what type of bones are indicative of a fat embolus?

long bones

84

What are the two gross manifestations of fat emboli?

dyspnea

petechiae on skin over chest

85

Pain from what two locations are characteristic of the bends?

joint and muscle

86

What happens to bones during Caisson disease?

multifocal ischemic necrosis of bone

87

Gestational emboli has what two histological components located inside of it?

squamous cells

keratin

88

What three veins does a PE most often arise from?

femoral, iliac and popliteal

89

What is elevated during a pulmonary embolism?

D-dimer

90

In what part of the heart do systemic emboli usually arise?

left heart

91

What is the first step of primary hemostasis?

transient vasoconstriction

92

What cell synthesizes TXA2 during the clottign cascade?

platelets

93

What organ is the most at risk of bleeding during severe thrombocytopenia?

brain

94

What is a normal bleeding time?

2-7 minutes

95

Which immunoglobulin can cross the placenta?

IgG

96

What cell undergoes hyperplasia during Immune Thrombocytopenia? Where?

Megakaryocytes

on bone marrow biopsy

97

What is the secondary Tx of immune thrombocytopenia?

IV immunoglobulin = secondary

gives splenic macrophages something to chew on rather than IgG anti-GpIIb/IIIa

98

What cell is depleted during Microangiopathic Hemolytic Anemia?

platelets

99

Where is E. coli O157:H7 found?

undercooked hamburger

100

What cell type is damaged that leads to HUS?

endothelial

101

Other than HUS, what can E. coli O157:H7 cause? What specific toxin?

Dysentery

verotoxin

102

What is the mnemonic to remeber the five clinical finding of TTP and HUS?

CRSMF

103

What does the C of CRSMF stand for?

CNS abnormalities

104

What does the R of CRSMF stand for?

renal insufficiency

105

What does the S of CRSMF stand for?

skin and mucosal bleeding

106

What does the M of CRSMF stand for?

Microangiopathic hemolytic uremia

107

What does the F of CRSMF stand for?

Fever

108

Why does TTP and HUS cause skin and mucosal bleeding?

low platelets due to microthrombi formation

109

What myeloid cell would increase in number on a PBS during TTP or HUS?

Megakaryocyte

110

Why are corticosteroids used to treat TTP?

corticosteroids decrease number of antibody producing cells

111

What do platelets look like in Bernard-Soulier syndrome?

enlarged

112

What drug can expose Glanzmann-Thrombasthenia? Why?

Aspirin

lack of TXA2 prevents aggregation

113

What cell contributes the phospholipid surface to the coagulation cascade?

platelets

114

What two locations body does a patient with Hemophilia A present with bleeding?

deep tissue

post-surgical

115

What is the treatment for Hemophilia A?

Recombinant Factor 8

116

How does von Willebrand Disease typically present?

mucosal and skin bleeding

117

What is the most common disorder of coagulation?

vWF disease

118

What coagulation factor does vWF bind to and stabilize?

eight

119

Regarding coagulation, what specific chemical reaction does Vitamin K catalyze?

gamma carboxylation

120

Regarding coagulation, what enzyme is located in the lilver?

Vitamin K epoxide reductase

121

What is the feared result of HIT?

thrombosis

122

What are the two common bleeding sites of DIS?

mucosal surfaces

IV sites

123

Regarding DIC, what in amniotic fluid can activate the coagulation cascade?

tissue thromboplastin

124

What specific type of cancer can cause DIC? Why?

acute promyelocytic leukemia

expansion of platelets and their release of primary granules

125

What type of histological feature is present in Acute Promyelocytic Leukemia?

Auer rods

126

What is the Tx for DIC? What is contained in this?

Cryoprecipitate

fibrinogen

127

What protein inactivates plasmin?

α2 anti-plasmin

128

What factor is used to differentiate a disorder of fibrinolysis vs. DIC?

fibrinolysis disorder = normal platelet count

DIC = low platelets

129

What cell type produces anti-Thrombin III?

endothelial cells

130

What cell type produces tPA?

endothelial

131

What cell type produces thrombomodulin?

endothelial cells

132

What are the three causes of endothelial damage?

atherosclerosis, vasculitis, high serum homocysteine

133

Initial warfarin therapy results in a deficiency of what two anti-coagulants?

Protein C and Protein S

134

What is the mutated version of prothrombin? What mutation is present?

20210A

point mutation

135

What is the key histological fidning of an atherosclerotic embolus?

cholesterol clefts

136

What is the chronic form of a gas embolus?

Caisson Disease

137

What is Caissons disease characterized by?

multifocal ischemic necrosis of bone

138

What is the main complication of chronic pulmonary emboli?

pulmonary hypertension

139

How can anemia present in an individual with already poor coronary vascular function?

angina

140

Where in the GI tract does absorption of ironn occur?

duodenum

141

What is contained within α-granules of platelets?

vWF and fibrinogen

142

Why can oral contraceptives result in a hypercoaguable state?

estrogen promotes synthesis of clotting factors

143

What type of neoplasia can cause iron deficient anemia?

colonic carcinoma

144

What is the most common cause of a decrease in ADAMTS13?

acquired autoantibody

145

Would a disorder of fibrinolysis present with D-dimers? Why or why not?

no

decreased fibrinolysis only--> no degradation of D-dimers to begin with

146

What is lysed during a disorder of fibrinolysis?

fibrinogen

147

What is the most common cause of thrombocytopenia in children and adults?

Immune Thrombocytopenia

148

What is the most common inherited coagulation disorder?

Bernard soulier disease

149

Why does Prothrombin 20210A result in a hyper-coaguable state?

too high of levels of prothrombin

150

Which granule of platelets is vWF contained?

α-granules

151

What is a normal bleeding time?

2-7 minutes

152

Which myeloid cell does uremia interrupt?

platelets

153

Which four clotting factors does the PT follow?

two

five

seven

ten

154

PTT follows all factors except which two?

seven and thirteen

155

Does HIT produce a hypocoaguable or hypercoaguable state?

hyper

156

Which two cytokines are involved during DIC? Via wat mechanism?

TNF and IL1

induce endothelial cells to release tissue factor

157

Does use of lytics increase or decrease BT?

increase

158

Which cell type synthesizes AT III?

endothelial

159

What does plasmin do to platelets?

blocks their aggregation

160

Which cell type secretes tPA?

endothelial

161

What gets transferred from THF to B12? What is the fate of this molecule?

methyl group

homocysteine to methionine

162

Is GpIb involved with adhesion or aggregation?

adhesion

163

Is GpIIb/IIIa involved with adhesion or aggregation?

aggregation

164

What is the key difference between DIC and disorders of fibrinolysis?

no D-dimer with fibrinolysis problems

165

What type of surgery can cause a gas emboli?

laproscopic