Hematology Flashcards

1
Q

What is the function of thrombin?

A

Key to coagulation
Converts fibrinogen to fibrin
Activates factors V and VIII
Activates platelets

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2
Q

What is the function of antithrombin III?

A

Key to anticoagulation
Binds and inhibits thrombin
Inhibits factors IX, X, and XI
Heparin activates antithrombin III

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3
Q

What is the function of protein C?

A

Degrades factors V and VIII

Degrades fibrinogen

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4
Q

What is the function of plasmin?

A

Degrades factors V and VIII, fibrinogen, and fibrin

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5
Q

What factor has the shortest half life?

A

Factor VII

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6
Q

What is the only factor not synthesized in the liver?

A

Factor VIII —synthesized in the endothelium with vWF

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7
Q

The activity of what factors are lost in stored blood, but NOT FFP?

A

Factor V and VIII

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8
Q

What factors are vitamin K dependent?

A

Factors II, VII, IX, X, protein C and S

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9
Q

What works faster, PO or IV potassium?

A

PO. IV K+ takes 12 hours to take effect

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10
Q

How long does it take FFP to work?

A

Immediately

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11
Q

What is factor II?

A

Prothrombin

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12
Q

What is the half life of RBC?

A

120 days

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13
Q

What is the half life of platelets?

A

7 days

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14
Q

What is the half life of PMNs?

A

1-2 days

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15
Q

What is the function of prostacyclin (PGI2)?

A

From endothelium
Decreased platelet aggregation
Causes vasodilation
Increases cAMP in platelets

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16
Q

What is the function of thromboxane?

A

From platelets
Increases platelet aggregation
Causes vasoconstriction
Triggers release of calcium in platelets —> exposes GpIIb/IIIa receptor to initiate platelet-platelet binding and platelet-collagen binding (GpIb)

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17
Q

What product has the highest concentration of vWF-VIII?

A

Cryoprecipitate

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18
Q

What product has high levels of fibrinogen?

A

Cryoprecipitate

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19
Q

What product has the highest levels of all coagulation factors, protein C and S, and AT-III?

A

FFP

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20
Q

What 2 things cause release of factor VIII and vWF from endothelium?

A

DDAVP and conjugated estrogen

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21
Q

What lab is best to measure liver synthesis function?

A

Prothrombin time (PT)—measures factors II, V, VII, and X, fibrinogen

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22
Q

What is the normal activated clotting time (ACT)?

A

150-200 sec for routine anticoagulation

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23
Q

What is the goal ACT for cardiopulmonary bypass?

A

> 480 seconds

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24
Q

At what INR is it a relative contraindication for performing surgical procedures?

A

> 1.5

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25
Q

At what INR is it a relative contraindication to central line placement, percutaneous needle biopsies, and eye surgery?

A

> 1.3

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26
Q

What is the MCC of surgical bleeding?

A

Incomplete hemostasis

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27
Q

What is the MC congenital bleeding disorder?

A

Von Wilebrand’s disease

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28
Q

What is the MC symptom of vWF disease?

A

Epistaxis

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29
Q

What is the function of vWF?

A

Links GpIb receptor on platelets to collagen

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30
Q

What is the inheritance pattern of types I, II, and III VWF disease?

A

Types I and II are autosomal dominant

Type III is autosomal recessive

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31
Q

What test is abnormal in vWF disease?

A

Ristocetin test = bleeding time
PT normal
PTT can be normal or abnormal

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32
Q

What is the dysfunction in type I vWF disease?

A

Reduced quantity of vWF

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33
Q

What is the treatment for type I vWF?

A

Recombinant VIII:vWF, DDAVP, cryoprecipitate

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34
Q

What is the dysfunction in type II vWF disease?

A

Defect in vWF molecule so it doesn’t work well

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35
Q

What is the treatment for type II vWF disease?

A

Recombinant VIII:vWF, DDAVP, cryoprecipitate

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36
Q

What is the dysfunction in type III vWF disease?

A

Complete vWF deficiency (rare)

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37
Q

What is the treatment for type III vWF disease?

A

Recombinant VIII:vWF , cryoprecipitate

DDAVP will not work because there is no vWF to be released

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38
Q

What is hemophilia A?

A

Factor VIII deficiency

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39
Q

How is hemophilia A inherited?

A

Sex link recessive

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40
Q

What is the MC symptom of hemophilia A?

A

Hemarthrosis

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41
Q

If a patient has hemophilia A, what should the levels of factor VIII be pre-op and post-op?

A

Pre-op, need levels to be 100%

Post-op, levels should be 80-100% for 10-14 days post-op

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42
Q

What test is abnormal in hemophilia A?

A

Prolonged PTT and normal PT

Follow PTT Q8H after surgery

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43
Q

Which factor crosses the placenta?

A

Factor VIII—this is why newborns with hemophilia A may not bleed at circumcision

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44
Q

What should you do if patient with hemophilia A has joint bleeding?

A

DO NOT ASPIRATE

Ice, keep joint mobile with range of motion exercises, factor VIII concentrate or cryoprecipitate

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45
Q

What should you do if patient with hemophilia A has epistaxis, intractable hemorrhage, or hematuria?

A

Recombinant factor VIII, cryoprecipitate

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46
Q

What is hemophilia B?

A

Factor IX deficiency. Also known as Christmas disease

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47
Q

How is hemophilia B inherited?

A

Sex-linked recessive

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48
Q

If a patient has hemophilia B, what should the levels be pre-op and post-op?

A

Need level 100% pre-op

Must keep 30-40% for 2-3 days after surgery

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49
Q

What test is abnormal in hemophilia B?

A

Prolonged PTT and normal PT

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50
Q

What is the treatment for hemophilia B?

A

Recombinant factor IX or FFP

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51
Q

What labs are abnormal in factor VII deficiency?

A

Prolonged PT and normal PTT

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52
Q

Treatment for factor VII deficiency?

A

Recombinant factor VII or FFP

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53
Q

What signs are usually present in platelet disorders?

A

Bruising, epistaxis, petechiae, and purpura

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54
Q

What is Glanzmann’s thrombocytopenia?

A

GpIIb/IIIa receptor deficiency on platelets (usually links fibrin between platelets)

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55
Q

What is the treatment for Glanzmann’s thrombocytopenia?

A

Platelets

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56
Q

What is Bernard Soulier syndrome?

A

GpIb receptor deficiency on platelets —cant bind to collagen

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57
Q

What is the treatment for Bernard Soulier syndrome?

A

Platelets

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58
Q

What can cause acquired thrombocytopenia?

A

H2 blockers, heparin

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59
Q

How do we define uremia?

A

BUN > 60-80

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60
Q

What is the effect of uremia on hemostasis?

A

Inhibits platelet function by inhibiting release of vWF from endothelium

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61
Q

What is the treatment of bleeding due to uremia?

A

Hemodialysis-first line
DDAVP for acute reversal
Cryoprecipitate for moderate to severe bleeding

62
Q

What causes heparin-induced thrombocytopenia (HIT)?

A

Thrombocytopenia due to anti-heparin antibodies (IgG heparin

63
Q

What signs would you expect to see in a patient with HIT?

A

Platelets <100, a drop in platelets >50% of admission levels, thrombosis while on heparin

64
Q

What kind of clot forms in HIT?

A

A white clot

65
Q

How do you diagnose HIT?

A

ELISA for heparin Ab’s

Serotonin release assay

66
Q

What is the treatment for HIT?

A

Stop heparin, start argatroban (direct thrombin inhibitor) for anticoagulation

67
Q

Should you give platelets in HIT?

A

NO! Increases the risk of thrombosis

68
Q

What lab values would be abnormal in disseminated intravascular coagulation (DIC)?

A

Decreased platelets
Low fibrinogen
High fibrin split products (high D-dimer)
Prolonged PT and PTT

69
Q

What often initiates DIC?

A

Tissue factor

70
Q

What is the treatment for DIC?

A

Treat the underlying cause

71
Q

How long before surgery should a patient stop taking ASA?

A

7 days before

72
Q

What lab value will be abnormal in a patient taking ASA?

A

Bleeding time

73
Q

MOA of ASA?

A

Inhibits cyclooxygenase in platelets and decreases TXA2 —> irreversibly inhibits COX.
Platelets have no DNA, so cannot resynthesize COX

74
Q

How soon should a patient stop clopidogrel (Plavix) before surgery?

A

7 days before surgery

75
Q

MOA of Plavix?

A

ADP receptor antagonist

76
Q

If patient on Plavix is bleeding, what should you do?

A

Give platelets

77
Q

How soon should Coumadin be stopped before surgery?

A

7 days before surgery. COnsider starting heparin while Coumadin wears off

78
Q

If patient on Coumadin starts bleeding, what should you do?

A

Give vitamin K and FFP

79
Q

What do you want platelet levels to be before surgery?

A

> 50,000

80
Q

What do you want platelet levels to be after surgery?

A

> 20,000

81
Q

During what surgery is urokinase released, causing thrombolysis?

A

Prostate surgery

82
Q

What is a Amicar and what is it used for?

A

Aminocaproic acid. Inhibits fibrinolysis by inhibiting plasmin

83
Q

Does no bleeding at circumcision rule out bleeding disorders?

A

No because can still have clotting factors from mother

84
Q

What is the most common congenital hypercoagulability disorder?

A

Factor V Leiden mutation

85
Q

What is factor V Leiden?

A

Defect on factor V that causes resistance to activated protein C

86
Q

What is the treatment for factor V Leiden mutation?

A

Heparin, warfarin

87
Q

What is the treatment for hyperhomocysteinemia?

A

Folic acid and vitamin B12

88
Q

What is the prothrombin gene mutation?

A

G20210A

89
Q

Treatment for prothrombin gene defect?

A

Heparin, warfarin

90
Q

Treatment for protein C and S deficiency?

A

Heparin, warfarin

91
Q

Does heparin work for patients with AT-III deficiency?

A

Heparin does not work in these patients

92
Q

What can cause AT-III deficiency?

A

Exposure to heparin

93
Q

What is the treatment for AT-III deficiency?

A

Recombinant AT-III concentrate or FFP (highest concentration of AT-III) followed by heparin then warfarin

94
Q

What is the treatment for dysfibrinogenemia, dysplasminogenemia?

A

Heparin, warfarin

95
Q

What causes polycythemia Vera?

A

Bone marrow overproduction

96
Q

What do we worry about in patients with polycythemia Vera?

A

Thrombosis

97
Q

What is the goal before surgery in patients with polycythemia Vera?

A

Keep Hct < 48 and platelets <400 before surgery

98
Q

What is treatment for polycythemia Vera?

A

Phlebotomy, ASA, hydroxyurea

99
Q

What are the symptoms of anti-phospholipid syndrome?

A

Thrombosis, loss of pregnancy

100
Q

Do all patients with anti-phospholipid antibody syndrome have SLE?

A

NO

101
Q

What causes anti-phospholipid antibody syndrome?

A

Antibodies to phospholipids including cardiolipin and lupus anticoagulant

102
Q

What lab values are abnormal in anti-phospholipid antibody syndrome?

A

Prolonged PTT —> BUT STILL HYPERCOAGULABLE

103
Q

How to diagnose anti-phospholipid antibody syndrome?

A

Prolonged PTT (not corrected with FFP)
Positive Russell viper venom time
False-positive RPR test for syphilis

104
Q

Treatment for anti-phospholipid antibody syndrome?

A

Heparin, warfarin

105
Q

What is the most common factor causing hypercoagulability?

A

Tobacco

106
Q

Name acquired hypercoagulability syndromes?

A

Malignancy, inflammatory states, IBD, infections, oral contraceptives, pregnancy, RA, post-op patients, myeloproliferative disorders

107
Q

When does warfarin-induced skin necrosis occur?

A

When placed on Coumadin without being heparinized first

108
Q

What causes warfarin-induced skin necrosis?

A

Due to short half-life of proteins C and S, which are first to decrease in levels compared with the procoagulation factors —> results in hyperthrombotic state

109
Q

What patients are especially susceptible to warfarin-induced skin necrosis?

A

Patients with protein C deficiency

110
Q

What are the key elements in the development of venous thrombosis?

A

Virchow’s triad

111
Q

What is Virchow’s triad?

A

Stasis, hypercoagulability, and endothelial injury

112
Q

What is the key element for development of arterial thrombosis?

A

Endothelial injury

113
Q

Why does a hypercoagulable state occur during cardiopulmonary bypass? How do we prevent it?

A

Due to factor XII (Haegman factor) activation. Give heparin to prevent

114
Q

What is the treatment for DVT after first, second third one? PE?

A

First: warfarin for 6 months
Second: warfarin for 1 year
Third or significant PE: warfarin for life

115
Q

What are the indications for IVC filter?

A

Contraindications to anticoagulation
PE while on anticoagulation
Free-floating IVC, Iloilo-femoral, or deep femoral DVT
Recent pulmonary embolectomy

116
Q

Should IVC be placed above or below renal veins?

A

Below

117
Q

What is the source if a patient has a PE with a filter in place?

A

SVC (upper extremity), IVC above the filter, or gonadal veins

118
Q

If your clinical suspension for a PE is high, what should you do?

A

Do not wait for the CT scan, give heparin bolus, unless there is a contraindication

119
Q

If patient with PE is in shock despite massive knot ropes and pressors, what should you do?

A

Go to the OR for open removal or angiography for suction catheter

120
Q

What is the most common region where PEs come from?

A

Ilio-femoral region

121
Q

In what instances is aminocaproic acid used?

A

Used in DIC, persistent bleeding following cardiopulmonary bypass, and thrombolytic overdoses

122
Q

What is the MOA of warfarin?

A

Prevents vitamin K-dependent decarboxylation of glutamic residues on vitamin-K dependent factors

123
Q

Name the 3 oral, direct thrombin inhibitors

A

Dabigatran (Pradaxa), apixaban (Eliquis), and rivaroxaban (Xarelto)

124
Q

In what patients are oral direct thrombin inhibitors used?

A

Patients with a. Fib NOT due to heart valve problem and in patients with DVT or PE

125
Q

How do sequential compression devices help prevent DVT?

A

Improve venous return and induces fibrinolysis with compression (release of tPA from endothelium)

126
Q

How does heparin work?

A

Binds and activates ATIII (1000x more activity) and increased neutralization of factors IIa and Xa

127
Q

What is used to reverse effects of heparin?

A

Protamine (binds directly to heparin)

128
Q

What is the half life of heparin?

A

60-90 minutes

129
Q

What is the goal PTT when a patient is on heparin?

A

60-90 seconds

130
Q

What are some long-term side effects of heparin?

A

Osteoporosis, alopecia

131
Q

How is heparin cleared?

A

By the reticuloendothelail system (spleen, macrophages)

132
Q

Which anticoagulant can be used during pregnancy, warfarin or heparin?

A

Heparin because it does not cross the placental barrier. Warfarin does cross placental barrier

133
Q

What do we worry about when giving protamine?

A

Cross reaction with NPH insulin or previous protamine exposure
In 1% of patient, they get protamine reaction (hypotension, bradycardia, and decreased heart function)

134
Q

What is the difference between low molecular weight heparin (enoxaparin) and unfractionated heparin?

A

Low molecular weight heparin has a lower risk of HIT and binds to antithrombin III but increased neutralization of JUST factor Xa —> not reversed with protamine

135
Q

What is argatroban?

A

Reversible direct thrombin inhibitor

136
Q

Where is argatroban metabolized?

A

In the liver

137
Q

What is the half-life of argatroban?

A

50 minutes

138
Q

When is argatroban often used?

A

In patients with HIT

139
Q

What is bivalirudin?

A

Reversible direct thrombin inhibitor

Also known as angiomax

140
Q

How is bivalirudin metabolized?

A

By proteinase enzymes in the blood

141
Q

What is the half life of bivalirudin?

A

25 minutes

142
Q

What is Hirudin?

A

Irreversible direct thrombin inhibitor

143
Q

Where is hirudin derived from?

A

Leeches

144
Q

WHat isn’t eh most potent direct inhibitor of thrombin?

A

Hirudin

145
Q

What is Ancrod?

A

Malayan pit viper venom that stimulates tPA release

146
Q

What are the 3 MC thrombolytics?

A

TPA, streptokinase, and urokinase

147
Q

How do thrombolytics work?

A

Activate plasminogen

148
Q

How do you follow effects fo thrombolytics?

A

Follow fibrinogen levels

Fibrinogen leaves <100 is associated with increased risk and severity of bleeding

149
Q

What are absolute contraindications to thrombolytics?

A

Active internal bleeding
Recent CVA or neurosurgery (<3 months)
Intracranial pathology
Recent GI bleeding

150
Q

What are major contraindications to thrombolytic use?

A

Recent surgery (<10 days), organ biopsy, or obstetric delivery, left heart thrombus, active peptic ulcer, recent major trauma, uncontrolled HTN, recent eye surgery

151
Q

What are minor contraindications to thrombolytic use?

A

Minor surgery, recent CPR, atrial fibrillation with mitral valve disease, bacterial endocarditis, hemostasis defects (renal or liver disease), diabetic hemorrhagic retinopathy, pregnancy