Blood Disorders and Products Flashcards

1
Q

What is the most common hereditary bleeding disorder

A

vWF disorder

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2
Q

clinical signs patient may show with vWF disorder

A
  • easy bruising
  • recurrent epistaxis
  • menorrhagia
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3
Q

List the types of classifications of vWF disease

A
  • Type 1 = partial quantitative deficiency, mildest: responds to DDAVP
  • Type 2A= dysfunction platelet adhesion: may respond to DDAVP
  • Type 2M= dysfunction platelet adhesion: may respond to DDAVP
  • Type 2B= increased platelet binding affinity: thrombocytopenia with DDAVP
  • Type 2N= decreased F VIII-binding affinity: often confused with hemophilia A
  • Type 3= severe deficiency; rarest, most severe, usually requires factor concentrates
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4
Q

Which types of vWF are non-responsive to DDAVP

A

type 2N and 3

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5
Q

What is the shotgun approach to treating vWF disease

A

cryo

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6
Q

PT and PTT value for vWF disease

A

normal, bleeding time is prolonged

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7
Q

Starting dose of DDAVP

A

0.3-0.8mcg/kg in 50mL saline OVER 15-20 MINUTES

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8
Q

When is peak effect of DDAVP and duration of action

A

peak 30min, 6-8hr DOA

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9
Q

How does DDAVP treat vWF disease

A

Synthetic analogue of vasopressin and stimulates the release of vWF by endothelial cells

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10
Q

What are the side effects of DDAVP

A
  • headache
  • rubor
  • hypotension
  • tachycardia
  • hyponatremia
  • water intoxication
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11
Q

What should be done to decrease the hyponatremia and water intoxication when giving DDAVP

A

restrict all fluids 4-6hrs after giving DDAVP, recheck sodium levels after

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12
Q

What changes are seen at Na levels 120 meq/L

A

confusion, restlessness, widening of QRS

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13
Q

What changes are seen at Na levels 115 meq/L

A

somnolence, nausea, elevated ST, widened QRS

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14
Q

What changes are seen at Na levels 110 meq/L

A

seizures, coma, Vtach/Vfib

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15
Q

Cryoprecipitate poses an increased risk for

A

viral infection

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16
Q

1 unit of cryo should raise fibrinogen levels by how much

A

50mg/dL

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17
Q

F VIII may be better than cryoprecipitate because it has less ____

A

risk for infection

contains both F VIII and vWF

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18
Q

When should DDAVP be given for surgery

A

60min prior to surgery

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19
Q

What should be done before surgery in patient with vWF disease

A
  • Consult hematology
  • Ensure normalization of bleeding time and improved levels of F VIII before surgery
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20
Q

Which anesthesia route should be used in patients with coagulopathies

A

General anesthesia

  • neuroaxial blocks have increased risk of developing hematoma and compression of neurological structures
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21
Q

Heparin is _____ charged

A

negatively

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22
Q

Heparin mechanism of action

A
  • inhibits thrombin (thrombin needed to convert fibrinogen to fibrin)
  • activating antithrombin III
23
Q

If a patient has no response to heparin they have _______

A

AT3 deficiency

24
Q

Which labs should be monitored with heparin

A

PTT and ACT

25
Normal ACT time
<150 seconds
26
Heparin reversal drug
protamine
27
Coumadin interferes with which factors
vitamin K- dependent factors : II, VII, IX, X
28
How long does it take vitamin K to reverse coumadin
6-8 hours
29
What can be given other than vitamin K to reverse coumadin faster?
prothrombin complex concentrates (expensive), recombinant factor VIIa, FFP, Cryo
30
How do fibrinolytics act
converting plasminogen to plasmin, which in turn cleaves fibrin, thereby causing clot dissolution.
31
What are 3 fibrinolytics
- tissue plasma activator (tPA) - streptokinase - urokinase
32
TXA is an
anti-fibrinolytic, keeps clots together
33
Which anti-fibrinolytic is available to give PO
tranexamic acid (TXA)
34
TXA dose
1-2 grams ped: 15-20mg/kg
35
What is a side effect of TXA
color vision loss
36
DIC is a result of what
systemic activation of the coagulation system simultaneously leads to thrombus formation and exhaustion of platelets and clotting factors
37
Clinical appearance of DIC looks like what
petechiae, loss of perfusion to areas, color change clots everywhere -> factors get depleted -> bleed everywhere
38
What are common underlying disorders that may precipitate DIC
trauma, amniotic fluid embolus, malignancy, sepsis, incompatible drug transfusions
39
What will labs show in DIC
- decreased platelets - increase PT/PTT - DIC panel = fibrin degredation products
40
Treatment of DIC includes
*treating underlying condition* - blood component transfusion to replete coag factors
41
What is contraindicated in DIC
antifibrinolytic therapy, treating the initial clotting can result in even worse bleeding once factors are depleted.
42
What score is used to evaluate sepsis and DIC
Apache
43
Which disorders are prothombotic
F V Leiden, HIT
44
Factor V Leiden is typically diagnosed when
pregnancy, frequent miscarriages
45
Factor V leiden occurs due to
Gene mutation for factor V - Factor V Leiden is an abnormal version of factor V that is resistant to the action of activated protein C -> activated protein C cannot easily stop factor V leiden from making more fibrin
46
Factor V Leiden is treated with
LMWH
47
Patients with factor V leiden are at higher risk for what
DVT, PE
48
How should surgery be timed around lovenox
10-12 hours after last dose, 4 hours before next dose
49
What is HIT
heparin induced thrombocytopenia - autoimmune-mediated drug reaction after exposure to unfractionated heparin (rarely LMWH) - results in platelet activation, potential for venous and arterial thromboses
50
How long after heparin does HIT present
thrombocytopenia 5-14 days after dose
51
What is the hallmark finding of HIT
platelets <100,000
52
Evidence suggests that HIT is mediated by ______
immune complexes (composed of IgG antibody, platelet factor 4, and heparin)
53
What should be done if HIT is suspected
STOP all heparin - give alternative anticoagulation such as bivalirudin, lepirudin, argatroban - fondaparinaux to treat VTE
54
AT3 Deficiency treatment
FFP