Coagulation Disorders Flashcards

1
Q

what are the VItamin K dependent factors?

A

II (prothrombin), VII, IX, X

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2
Q

what are the thrombin sensitive factors

A

I (fibrinogen), V, VIII, XIII

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3
Q

what is the Regulatory mechanism for maintaining blood flow

A

fibrinolytic system

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4
Q

when is plasminogen release?

A

in response to thrombin, venous stasis, exercise, ischemia

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5
Q

what is the extrinsic clotting pathway? Time to fibrin formation after addition of thromboplastin to plasma. Used w/ INR

A

PT- Prothrombin time

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6
Q

what is INR

A

International normalized ratio

ratio of PT patient/ PT normal

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7
Q

what cause elevated INR?

A

Liver disease
Vit K deficiency
Warfarin

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8
Q

what is a normal INR

A

0.8-1.2

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9
Q

what is aPTT

A

activated partial thromboplastin time

intrinsic and common pathways

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10
Q

When is aPTT elevated?

A

Liver dz
heparin
hypercoaguability

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11
Q

what does D-dimer tell you?

A

that plasma is working

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12
Q

when is a D-dimer positive?

A

DVT/PE

Disseminated intravascular coagulation (DIC)

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13
Q

what does heparin activate?

A

antithrombin III

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14
Q

what is the most common congenital bleeding disorder?

A

Von Willebrand Disease

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15
Q

What do patients w/ Von Willebrand Dz have decreased quantity of?

A

Von Villebrand factor

which stabilizes Factor VIII

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16
Q

What pathway is affected by VOn Willebrand Dz

A

intrinsic

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17
Q

what are the three types of Von Willebrand Dz

A

Mild to Moderate (most common)
Functionally abnormal
Nearly undetectable (most severe)

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18
Q

what is acquired VW dz associated w/?

A

autoimmune dz

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19
Q

what drugs are associated w/ acquired VW dz?

A

valproic acid
griseofulvin
hydroxyethyl starch (volume expander)
ciprofloxacin

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20
Q

S/S of VW dz

A

mucocutaneous bleeding
easy bruising
post-op bleeding

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21
Q

what lab will be abnormal w/ VW dz?

A
aPTT will be prolonged
VWF low 
(PT and platelets are w/i normal range)
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22
Q

tx for VW dz?

A

apply topical thrombin (surgery)

GIve exogenous VW factor or Factor VIII

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23
Q

What drug can be given for VW dz?

A

Desmopressin (DDAVP)

stimulates release of VFW and factor VIII

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24
Q

ADRs of desmopressin

A

tachycardia
HA
flushing

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25
Q

what to monitor when giving desmopressin

A

pulse, BP
Factor VIII levels
aPTT
bleeding time

26
Q

what are antifibrinolytic therapies for VW dz? Often used as an adjunct

A
aminocaproic acid
tranexamic acid (surgical preps, for non coag problems)
27
Q

what do you need to use caution w/ for aminocaproic acid and tranexamic acid?

A

Renal dysfunction

28
Q

ADRs of aminocaproic acid and tranexamic acid?

A

Diarrhea
N/V
HPOTN
Thrombosis/ DVT

29
Q

what is used in patients unresponsive to DDAVP?

A

WVF/Factor VIII concentrates

30
Q

ADRs of VWF/ factor VIII concentrates

A

thrombosis/ DVT
chills, HPOTN, HA, edema
viral infection (chance for infection from human blood products)
anaphylaxis

31
Q

Hemophilia A means patients lack what?

A

Factor VIII

32
Q

Hemophilia B means patients lack what?

A

Factor IX

33
Q

S/S of hemophilia

A

palpable ecchymoses
Hemarthroses (joint pain, swelling, decreased ROM)
muscle hemorrhage
hematuria

34
Q

what labs will be abnormal w/ hemophilia?

A

prolonged aPTT
reduced factor VIII or IX level
will have normal VW

35
Q

when should you test for hemophilia?

A

any male w/ unusual bleeding

36
Q

what vaccines do patients receiving blood factors need?

A

Hep A and B

37
Q

what can be used for mild bleeding in those w/ hemophilia A?

A

DDAVP

38
Q

what can you use an an adjunct for prevention of bleeding w/ dental procedures w/ hemophilia.

A

aminocaproic acid

tranexamic acid

39
Q

any patient less than 2 w/ hemophilia and no prior bleeding events what do you need to do?

A

primary prophylaxis

40
Q

once patients are over 2 w/ hemophilia and don’t have bleeding episodes what do you?

A

primary prophylaxis

41
Q

if a patient w/ hemophilia has a bleed what do you need to do?

A

secondary prophylaxis

42
Q

what’s the difference b/w primary and secondary prophylaxis

A

dosing of drugs

43
Q

what virus is seen in recombinant factor VIII?

A

parovirus (comes from human cell line)

44
Q

Does factor IX come from a human cell line?

A

No

45
Q

do plasma derived factor or recombinant work better for hemophilia B?

A

plasma, but better to use recombinant factors because there is less risk

46
Q

what are some ADRs w/ infusable factors

A

Chills, fever, HPOTN, edema
thrombosis/ DVT
anaphylaxis
developing antibodies

47
Q

if developing antibodies more common in hemophilia A or B?

A

hemophilia A

48
Q

when should you suspect that the patient has produced IgG antibodies

A

post-infusion factor levels are lower than predicted

49
Q

if you can’t overcome the anitbodies produced by person, what must you give?

A

concentrated activated factors

50
Q

what is prothrombin compelx concentrates (PCC)

A

plasma derived

contains Vit K dependent factors

51
Q

what is used for bleeding episodes or prophylaxis before surgery.
can’t monitor effect w/ labs

A

Recombinant activated factor VII

52
Q

what other meds do hemophiliacs need?

A

pain meds

intraarticular dexamethasone for joint pain

53
Q

Acquired homeostatic disorder
Due to underlying condition or illness
Inappropriate systemic thrombosis followed by massive bleeding

A

Disseminated Intravascular Coagulation (DIC)

54
Q

what are the three things that happen w/ DIC

A

hypercoagulability –> widespread inflammation
thrombosis (consumes platelets and coags)
bleeding

55
Q

what are S/S of DIC?

A
Bleeding/oozing
Thrombosis
Petichiae/purpura
Peripheral cyanosis
Gangrene of distal extremities
Hemorrhagic bullae
56
Q

what labs w/ be abnormal w/ DIC (check serially)

A
Elevated PT and aPTT
thrombocytopenia
elevated D-dimer and fibrin degradation products (FDP)
decreased antithrombin III
decreased proteins C and S
decreased fibrinogen
end organ damage
57
Q

Tx for DIC (bleedign stage)

A

platelet infusion
Fresh Frozen Plasma (FFP)
Cryoprecipitate (can be used to replace fibrinogen)

58
Q

patients with what dz can look like DIC

A

liver dz (but this has normal fibrinogen where DIC doesn’t)

59
Q

what drugs do you use for a patient in DIC in thrombotic phase?

A

heparin or LMWH

60
Q

when can you consider antifibrinolytics w/ DIC

A

phase of fibrinolysis/ bleeding

61
Q

what can you not co-administer w/ antifibrinolytics

A

heparin

62
Q

what are the activated factors?

A

Prothrombin complex concentrates (PCC)

recombinant activated factor VII