Hematology I Flashcards

1
Q

The intima (the inner layer)

A

it is made up primarily of endothelial cells. The smooth endothelial lining physically repels the blood components away from the vessel wall, preventing activation of the clotting mechanism.

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2
Q

The second layer of the vessel wall

A

extremely thrombogenic and very active.

contains:
Collagen, a potent and important stimulus for platelet attachment to the injured vessel wall
Fibronectin, which facilitates the anchoring of fibrin during the formation of a hemostatic plug.

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3
Q

The third layer, the adventitia/externa

A

, participates in the control of blood flow by influencing the vessel’s degree of contraction.

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4
Q

Platelets

A

Platelets are round and disklike and circulate freely within the blood.

Platelets are constantly working to “patch” thousands of minute vascular injuries that occur in perpetuity. Approximately 7.1 × 103are used each day.

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5
Q

Where are platelets formed?

A

They are formed in the bone marrow from megakaryocytes, maintain a concentration count of approximately 150,000 to 300,000/mm3

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6
Q

how long do platelets live?

A

8-12 days

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7
Q

how do platelets flow?

A

The platelets flow along the vessel surface. Because they are smaller than some other constituents in fluid blood (e.g., RBCs, WBCs), they tend to be pushed aside, strategically positioned near the vessel-wall surface where they can then “react” in the event of injury.

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8
Q

Platelet function

A

Platelets are largely inactive unless they become activated as a result of vascular trauma. Adequate hemostasis is not possible in the absence of an adequate quality or quantity of activated platelets. Platelets work in conjunction with plasma proteins of the coagulation cascade to build a stable clot when injury to the vascular integrity occurs.

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9
Q

traditional description of clot formation in the response to injury

A

adherence of the platelet to the injured vessel wall and the response of the clotting cascade to form a stable clot and stop the progress of bleeding

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10
Q

What happens immediately after injury?

A

The vessel wall immediately contracts to cause a tamponade, decreasing blood flow.

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11
Q

3 phases of formation of primary plug

A

adhesion, activation, and aggregation

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12
Q

adhesion stage

A

vWF mobilizes from within the endothelial cells and emerges from the endothelial lining. Glycoprotein Ib (GpIb) receptors emerge from the surface of the platelet. The purpose of GpIb is to attach to vWF and attract platelets to the endothelial lining; vWF makes platelets “sticky” and allows them to adhere to the site of injury

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13
Q

Activation stage

A

platelet then undergoes a conformational transformation as it becomes activated under the influence of TF (a cofactor of the extrinsic clotting pathway)

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14
Q

aggregation phase

A

platelets form a mound whose only goal is to seal and heal the site of injury within the blood vessel.

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15
Q

Cofactors

A

Most cofactors are enzymes, with some exceptions (e.g., factors V and VIII).
The coagulation factors circulate as inactive cofactors until they are activated to assist in the process of coagulation.
Activation of cofactors results from either tissue or organ damage and sets in motion a process that terminates in stabilization of hemorrhagic conditions in the absence of pathology.

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16
Q

where is von wildebrand factor synthesized

A

endothelial cells

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17
Q

factors dependent on Vit K?

A

2, 7, 9,10

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18
Q

describe the intrinsic coagulation pathway

A

damage to vessel (internal) –> factor XII –> Factor XIIa –> Factor XI –> Factor XIa –> Factor IX –> Factor IXa; Factor IXa and Factor VIII come into the common pathway

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19
Q

describe the extrinsic coagulation pathway

A

external tissue damage causes conversion of Factor VII to factor VIIa, that along with tissue factor enter the common pathway

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20
Q

Thrombin (Factor IIa) assists in activating what factors? and influences recruitment of ______________ to the injured area w/ coagulation

A

I, V, VIII, XIII; platelets

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21
Q

T/F: most coagulation proteins are synthesized in the spleen

A

false; liver

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22
Q

which clotting factors are vitamin K dependent for utilization?

A

II, VII, IX, and X

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23
Q

which factor of the clotting cascade is required to ensure the platelet plug will hold, d/t it helping to form a cross linked mesh within the platelet plug

A

XIII

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24
Q

with the H&P what important questions would you ask directly related to issues with bleeding?

A
  1. do you experience any unusual bleeding or bruising (gums, nose bleeds, stools)
  2. hx of previous bleeding with dental procedures?
  3. repeated spontaneous bleeding episodes or excess bleeding after trauma?
  4. hx of bleeding more than anticipated in surgery?
  5. Family hx of bleeding tendencies/d/o?
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25
Q

H&P related to bleeding

A

1.important questions to ask r/t bleeding
2. do you see any physical signs of bleeding? bruising/petechiae
3. do they have any disorders of malnutrition, liver insufficiency, or possible vitamin D deficiency
4. pre-existing inherited coagulation d/o
5. preoperative use of medications that may influence bleeding/clotting

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26
Q

why would you be concerned about bleeding in a patient with: liver disease, cirrhosis, or absorption issues?

A

it would impair vitamin K synthesis and therefore the synthesis of vitamin K dependent clotting factors: II, VII, IX, X

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27
Q

anticoagulation medications

A

heparins and heparin derivatives
coumadin and derivatives
direct thrombin inhibitors

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28
Q

procoagulation medictions

A

vitamin K

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29
Q

antiplatelet meds

A

NSAIDs
ASA
persantine
thienopyridine

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30
Q

antifibrinolytic medications

A

amicar
transexamic acid

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31
Q

nonherbal dietary (vitamins) medications that can disrupt or influence coagulation

A

Vitamin K and E
Coenzyme Q10
Zinc
Omega 3 fatty acids

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32
Q

Herbals that can influence coagulation

A

garlic
ginger
ginko
feverfew
fishoil
flaxseed oil
black cohosh
cranberry

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33
Q

what are the most frequently assessed tests related to bleeding d/o?

A

Pt and aPTT

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34
Q

What do PT and APTT measure?

A

platelet function: adhesion and aggregation

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35
Q

T/F: modest prolonged bleeding time (PT and aPTT) does not predict surgical bleeding

A

true

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36
Q

what meds can alter bleeding time

A

NSAIDs and ASA

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37
Q

T/F: a normal platelet count inadvertently means they are functioning correctly

A

false; only tells how many are in plasma does not give information on fx

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38
Q

normal plt count

A

150,000 - 300,000

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39
Q

plt count is used to evaluate what

A

pts with petechiae , unexplained spontaneous bleeding, and to monitor thrombocytopenia

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40
Q

what is the primary role/fx of plt

A

maintain vascular integrity
aggregate when plug is needed to stop bleeing
help initiate clotting cascade

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41
Q

thrombocytopenia is defined as

A

< 150,000

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42
Q

surgical risk for bleeding would be when plts are < ___________

A

50,000

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43
Q

at what plt count is a pt at risk for spontaneous bleeding

A

< 20,000

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44
Q

normal PT

A

12-14 seconds

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45
Q

PT will be prolonged with d/o of the ______________ and ____________ pathways

A

extrinisic; common

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46
Q

coumadin and derivatives will alter which diagnostic bleeding time lab value

A

PT

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47
Q

normal INR

A

0.8-1.2

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48
Q

where do you want a pts INR before they go to surgery

A

1.2

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49
Q

normal aPTT

A

25-32 seconds

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50
Q

aPTT will be prolonged in disorders of the _____________ and ____________ pathways

A

intrinsic and common

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51
Q

aPTT is altered by what meds

A

heparin and LMWH

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52
Q

we use aPTT to monitor anticoagulation with _______________ therapy

A

heparin

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53
Q

we do not see evidence of prolonged PT or aPTT until it is decreased by ___________%

A

30

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54
Q

normal activating clot time (ACT)

A

90-150seconds

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55
Q

for cardiac surgery you would like to see an ACT of >

A

> 400sec

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56
Q

what is a TEG

A

measures the process of clot formation over time

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57
Q

what is the benefit of a TEG

A

allows you to evaluate platelet reactions, coagulation, and fibrinolysis over time

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58
Q

results of a TEG provide an indication of

A

clot strength
plt number and fx
intrinsic pathway defects
thrombin formation
rate of fibrinolysis

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59
Q

____________________ is used to guide blood product administration

A

TEG

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60
Q

T/F: you can use TEG to determine disorders in any coagulation pathway

A

false; INTRINSIC only

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61
Q

_________________ is a protein synthesized by endothelial cells and megakaryocites

A

von willebrand factor

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62
Q

what is von willebrand disease

A

failure to synthesize or secrete vWF or an accelerated clearance of vWF.
rare
may be inherited or acquired
sx range from mild to severe

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63
Q

when would you consider someone to have vWD?

A

when vWF is <30% of normal

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64
Q

which category of vWB would you have no evidence of regular/spontaneous bleeding, but bleeding may be likely after a surgery or trauma

A

mild

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65
Q

which category of vWB would you have spontaneous bleeding which can be relentless

A

severe

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66
Q

what is the most common inherited subtype of vWB

A

type 1 mild

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67
Q

acquired vWD is associated with…..

A

malignancy
autoimmune disorders
hypothyroidism

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68
Q

pt presents with mucocutaneous bleeding, and no clinical family hx of bleeding, but has hypothyroidism. What would you be concerned with

A

acquired vWD

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69
Q

what is the tx of vWD?

A

DDAVP
Tranexamic acid (Txa)
Factor VIII/vWF concentrate or cryoprecipitate

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70
Q

what is the drug of choice in tx’ing vWD

A

DDAVP

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71
Q

if you do not have Factor VIII/vWF concentrate in an acute bleeding situation in pt with vWD, what can you use?

A

cryoprecipitate

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72
Q

what do you give in an acute bleeding situation in a patient with vWD?

A

Factor VIII/vWF concentrate or cryopreciptate
avoid all antiplatelet drugs

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73
Q

___________________ is a x-linked hematologic recessive d/o characterized by unpredictable bleeding patterns

A

hemophilia

74
Q

T/F: hemophilia affects males almost exclusively

A

true

75
Q

hemophilia A is a deficiency of factor ___________, and B is a deficiency of factor _____________

A

VIII; IX

76
Q

people with this bleeding disease often exhibit spontaneous bleeding, muscle hematomas, and joint pain leading to progressive arthropathy and orthopedic surgical intervention d/t severely decreased range of motion

A

hemophilia

77
Q

mild hemophila A has factor VIII levels about _____________% to normal

A

5-30

78
Q

moderate hemophilia A has factor VIII levels of ______________% to normal

A

1-5

79
Q

moderate hemophilia A has factor VIII levels of ______________% to normal

A

<1

80
Q

which category of hemophilia A is most common

A

severe

81
Q

what is referred to as Christmas disease

A

hemophilia B (factor IX def)

82
Q

T/F: hemophilia A and B are clinically indistinguishable from one another

A

true

83
Q

considerations if pt comes in for surgery with hx of hemophilia

A
  1. hematology consult
  2. detailed coagulation profile
  3. detailed factor replacement plan prior to day of surgery
  4. plan for how to monitor levels in the perioperative period
84
Q

tx for hemophilia A

A

cryoprecipitate
DDAVP
TXA and amicar
Factor VIIa

85
Q

tx for hemophilia B

A

FFP
TXA amicar
Factor VIIa

86
Q

________________ is a hemostatic agent of last resort for pts with hemophila A and B

A

Factor VIIa

87
Q

_____________ reduces the efficacy of Factor VIIa (used to tx hemophilia A/B)

A

acidosis

88
Q

T/F: DDAVP is a treatment for hemophilia A and B

A

false; no value in the tx of hemophilia B. used in Hemophila A

89
Q

_______________ plays a central role in the development of DIC

A

tissue factor

90
Q

primary cause of acute DIC

A

sepsis

91
Q

what plays a role in the development of chronic DIC

A

tumors and large aortic aneurysms

92
Q

how do you dx DIC

A

clinical presentation with laboratory tests such as: plt, aPTT, PT, fibrinogen, antithrombin, D-dimer

93
Q

how do you Tx DIC

A
  1. ID and tx underlying cause
  2. hemodynamic support if needed
  3. blood products
  4. heparin
94
Q

when would you use heparin in the tx of DIC

A

for DIC manifested by thrombosis or acrocyanosis and without active bleeding

95
Q

what blood products could you give in the tx of DIC with active bleeding or high risk for bleeding

A

FFP
plts
cryoprecipitate and Antithrombin III

96
Q

what is the most commonly inherited thrombophilia ?

A

factor V leiden

97
Q

this is an autosomal dominant mutation that causes activated protein C resistance

A

Factor V leiden

98
Q

how do you manage factor V leiden thrombophilia

A

antithrombotic therapy

99
Q

anemia is described as a hgb [ ] of less than ______ in women and _____ in men

A

12; 13

100
Q

what is THE most important adverse effect with anemias

A

reduction in arterial O2 concentration and potential decreased delivery to tissues.

101
Q

H&P for anemia

A

general appearance
functional capcity
do they have fatigue
hx of blood transfusion?
bone pain?
splenectomy?

102
Q

Dx testing for anemia

A

CBC
Iron and TIBC
type and screen/type and cross

103
Q

_________________ is an autosomal recessive disorder for abnormal hgb, Hgb S

A

sickle cell anemia

104
Q

if heterozygous for the autosomal recessive hgb S you have ______________; if homozygous you have __________________

A

sickle cell trait
sickle cell disease

105
Q

which is more common heterozygous Hgb S (sickle cell trait) or homozygous Hgb S (Sickle cell disease)

A

heterozygous; sickle cell train ~ 10% of AA

106
Q

those with sickle cell trait (heterozygous) you may not see sickling until PaO2 is ___________, but with sickle cell disease (homozygous) you may see it with a PaO2 of ________________

A

20-30; 30-40

107
Q

triggers for sickle cell crisis

A

hypoxemia
hypothermia
infection
dehydration
venous stasis
acidosis

108
Q

S/Sx of sickle cell crisis

A

chronic hemolytic anemia
intermittent vaso-occlusion
severe pain
end organ damage

109
Q

what is the most common surgical procedure those with Sickle cell disease have to have? and why?

A

cholecystectomy; for gall stones d/t rapid breakdown of sickled RBCs –> increased bilirubin

110
Q

how do you manage sickle cell

A

adequate hydration
PRBC transfusion
avoid hypoxemia
maintain normothermia
maintain normal acid base balance
adequate perioperative pain managemetn
caution with vaso-occlusive devices (tourniqutes)

111
Q

for pt with sickle cell coming in for surgery; when would you consider giving PRBC peroperatively?

A

for young pts that are low to mod risk
to decreased Hgb S level to < 30-50%
for those that PRBC will not cause hgb to become > 10-11

112
Q

this is a group of x-linked disorders resulting in RBC hemolysis when exposed to oxidative stress; is d/t a deficiency in _______________________

A

glucose-6 phosphate dehydrogenase

113
Q

this disorder can present in african, middle eastern, mediterranean, and asian populations. may have severe newborn jaundice, acute hemolytic episodes throughout life, but most are asymptomatic throughout life. what is this?

A

glucose-6-phosphate dehydrogenase deficiency

114
Q

in glucose-6-phosphate dehydrogenase deficiency, what oxidative stressors could lead to RBC hemolysis

A

infection
hypothermia
fava beans
meds: antimalarials, sulfonamides, nitrofurantoin, ciprofloxin, methylene blue, antipyretic analgesics

115
Q

_______________ decribes an abnormally high Hct

A

polycythemia

116
Q

what is the polycythemia that is due to reduction in plasma volume without an increase in red cell mass?

A

relative polycythemia

117
Q

fasting would cause what type of polycythemia

A

relative polycythemia; reduction in plasma volume without increase in red cell mass

118
Q

in polycythemia a HCT of > 55-60% causes what?

A
  1. increases whole body viscosity which affects blood flow esp in small vessels and cerebral circulation
  2. increase risk for thrombosis arterial and venous
119
Q

thrombocytopenia is defined as a plt count less than _____________________

A

150,000

120
Q

what are the causes of thrombocytopenia?

A
  1. decreased production: bone marrow, folate deficiency, and/or malignancy
  2. sequestration - liver and spleen diseases
  3. increased destruction: sepsis, DIC, ITP, TTP
121
Q

T/F: risk of bleeding is inversely related to plt count

A

true

122
Q

Generally, you can go to surgery if plt is greater than ______________

A

50,000

123
Q

what is heparin induced thrombocytopenia?

A

immune response to heparin where plts are destroyed to > 50% decrease in plt count from baseline or plt < 100,000 from normal baseline count

124
Q

complications of HIT

A

severe thrombosis which can lead to amputation and/or death

125
Q

this HIT type occurs 1-4 days after start of heparin therapy

A

Type I HIT

126
Q

HIT type I is __________________ mediated and type II is ________________ mediated

A

non-immune; immune

127
Q

T/F: with HIT type I the thrombocytopenia will resolve spontaneously even with continued administration of heparin

A

true

128
Q

T/F: HIT type I is associated with increased risk of thrombosis and serious complications

A

false

129
Q

which type of HIT has an onset 5-14 days after the start of heparin therapy, and has severe thrombocytopenia affiliated with thrombosis and severe complications

A

HIT type II

130
Q

Hit type __________ induces a clinically relevant hypercoagulable state with clotting and thrombus formation

A

II

131
Q

which type of HIT does not resolve spontaneously, and requires the heparin therapy to be stopped to resolve?

A

type II

132
Q

how do you diagnose HIT

A

thrombocytopenia
cutaneous abnormalities and tachyphylaxis after heparin administration

133
Q

tx of HIT

A
  1. consult hematology
  2. stop heparin even prior to confirmatory lab tests
  3. administer direct thrombin inhibitors: Argatroban or lepirudin
  4. treat complications of thrombus formation
134
Q

what is the leading cause of morbidity and mortality after orthopedic surgery

A

thromboembolic events

135
Q

what ortho surgeries are the highest risk for thromboembolic events?

A

Total hip arthroplasty
total knee arthorplasty
pelvic fractures

136
Q

Triad etiology behind thromboembolic events

A

triad: venous stasis, hypercoagulability, and vascular trauma

137
Q

for total hip/knee patients should recieve LMWH when?

A

either > 12 hours preop or > 12 hours post op

138
Q

those undergoing a major orthopedic surgery esp for total hip or total knee should recieve what post op prophylactic therapy to reduce risk of thromboembolic event?

A

LMWH or compression device for minimum of 10-14 days

139
Q

postop prevention of DVT

A

early mobilization or ambulation
compression stockings
intermittent pneumatic compression (SCD)

140
Q

ASA should be d/c’d how many days prior to surgery

A

7

141
Q

ASA is antiplatelet which works by inhibiting _________________, and is ________________

A

cox-1; irreversible

142
Q

NSAIDs are antiplatelets which inhibit __________________, but is _______________

A

Cox-1 or 2; reversible

143
Q

how do you reverse NSAIDs?

A

platelets

144
Q

most Cox-1 inhibitors (NSAIDs) should be d/c’d how many days prior to surgery

A

3

145
Q

T/F: selective Cox-2 inhibitors are okay to continue up until the day of surgery

A

true

146
Q

how many days before surgery should Thienopyridine derivative (antiplt) meds like plavix and ticlid be d/c’d?

A

7-10 days prior

147
Q

Abciximab (Eliquis) should be d/c’d ______________ before surgery

A

48-72 hours

148
Q

Low-molecular weight heparin should be d/c’d ______________ before surgery

A

12-24 hours

149
Q

how do you reverse LMWH and heparin?

A

protamine sulfate

150
Q

heparin should be d/c’d _______________ before surgery

A

6 hours

151
Q

how do you reverse coumadin (warfarin)

A

vitamin K
FFP
Factor VII

152
Q

warfarin (coumadin) should be d/c’d __________________ before surgery

A

5 days

153
Q

INR < ____________ okay for most surgery’s, but for those where blood loss can be a good issue want the INR at least ______________

A

1.5; 1.2

154
Q

if a patient is on coumadin, but they have 3+ risk factors for thromboembolus, how should you proceed before surgery?

A

bridge them to heparin or LMWH; coumadin must be stopped 5 days prior to surgery but heparin only 6 hours and LMWH only 12-24

155
Q

what would be considered risk factors for thromboembolism

A

Afib
CHF
rheumatic heart disease
TIA
DM
HTN
> 75 years of age
mechanical heart valves.

156
Q

what medications that we give in anesthesia account for the largest number of type 1 hypersensitivity reactions?

A

NMB

157
Q

list the NMB from most likely to cause type 1 hypersensitivity reaction to the least likely

A

succ > vec > roc > pancuronium > Cisatracurium

158
Q

what mediates a type I hypersensitivity reaction?

A

IgE

159
Q

sx of type I hypersensitivity reaction

A

mild cutaneous sx
bronchospasm
cardiopulmonary collapse and death

160
Q

T/F: 60-70% of anesthesia related reactions are IgE mediated

A

true

161
Q

algorithm of intraoperative anaphylaxis treatment

A
  1. d/c triggering agent, put in trendelenburg, give 100% FiO2
  2. Epi
  3. NS or LR 10-30 ml/kg
  4. secondary treatment meds: vaso, albuterol, BB, benadryl, or hydrocortisone
162
Q

Grade II anaphylaxis you administer what dose of epi?

A

10 -20 mcg SC/IM, IV

163
Q

Grade III anaphylaxis you administer what dose of epi?

A

100-200 mcg SC/IV/IM q1-2 min; 1-4 mcg/min

164
Q

Grade IV anaphylaxis you administer what dose of epi

A

1 mg IV, repeat as needed: 0.05-0.1 mcg/min

165
Q

in the secondary treatment phase of the intraoperative anaphylaxis algorithim, if the pt is unresponsive to epi, you should give what?

A

vasopressin 2-10 units IV

166
Q

secondary tx of intraoperative anaphylaxis if pt is still having bronchospasms what should you administer?

A

albuterol/ipratropium inhalants, or terbutaline SC

167
Q

secondary tx of intraoperative anaphylaxis that we think could be due to a preoperative beta blocker, we would give

A

glucagon 1-5 mg IV q5 min

168
Q

secondary treatment of intraoperative anaphylaxis d/t continued airway edema, you would give

A

hydrocortisone 100-250 mg IV

169
Q

if you have an intraoperative anaphylactic event, post resuscitation, how should you proceed?

A
  1. trend serum tryptase for 120 min
  2. 24 hour monitoring for recurrence
  3. notify family/pt of rxn
  4. referral to allergist
170
Q

factors that play a contributing role to the perioperative immune system dysfunction

A
  1. surgery
  2. blood transfusions
  3. hyperglycemia
  4. hypothermia
  5. general anesthesia
  6. opioids
171
Q

Surgical site infection is defined as….

A

an infection at or near surgical incision within 30 days of the procedure or within 1 year after implantation of prosthetic device

172
Q

which part of the immune system is activated during surgery?

A

innate

173
Q

d/t immunosuppression affiliated with surgery and anesthesia, what can occur postoperatively?

A

surgical site infection/sepsis
tumor metastasis

174
Q

T/F: surgical excision of a tumor may stimulate proliferation and metastasis of tumor cells

A

true

175
Q

which anesthetic meds actually reduces cancer recurrence and metastasis?

A
  1. local anesthetics
  2. ASA
  3. NSAIDs
176
Q

which anesthetic meds promote cancer progression and reduce long term survival

A

opoids

177
Q

T/F: pts given 80% oxygen postoperatively have a shorter cancer free survival period

A

true

178
Q

which anesthesic meds have no effect on cancer survival

A

NO and dexmethasone

179
Q

T/F: you should avoid volatile anesthetic agents in cancer surgery d/t reproliferation and metastasis of cancer cells

A

false; insufficient evidence to avoid these in surgery

180
Q

anesthesia management of the immunocompromised pt

A
  1. strict aseptic technique and maximum barrier precautions
  2. general or regional anesthesia or combined technique
  3. prophylactic abx administered 30 min prior to incision
  4. active warming measures to avoid hypothermia
  5. perioperative hyperglycemia must be avoided
  6. leukocyte poor and irridated blood products should be used when transfusion is unavoidable
  7. postoperative multimodal pain management.