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
what to monitor when giving desmopressin
pulse, BP Factor VIII levels aPTT bleeding time
26
what are antifibrinolytic therapies for VW dz? Often used as an adjunct
``` aminocaproic acid tranexamic acid (surgical preps, for non coag problems) ```
27
what do you need to use caution w/ for aminocaproic acid and tranexamic acid?
Renal dysfunction
28
ADRs of aminocaproic acid and tranexamic acid?
Diarrhea N/V HPOTN Thrombosis/ DVT
29
what is used in patients unresponsive to DDAVP?
WVF/Factor VIII concentrates
30
ADRs of VWF/ factor VIII concentrates
thrombosis/ DVT chills, HPOTN, HA, edema viral infection (chance for infection from human blood products) anaphylaxis
31
Hemophilia A means patients lack what?
Factor VIII
32
Hemophilia B means patients lack what?
Factor IX
33
S/S of hemophilia
palpable ecchymoses Hemarthroses (joint pain, swelling, decreased ROM) muscle hemorrhage hematuria
34
what labs will be abnormal w/ hemophilia?
prolonged aPTT reduced factor VIII or IX level will have normal VW
35
when should you test for hemophilia?
any male w/ unusual bleeding
36
what vaccines do patients receiving blood factors need?
Hep A and B
37
what can be used for mild bleeding in those w/ hemophilia A?
DDAVP
38
what can you use an an adjunct for prevention of bleeding w/ dental procedures w/ hemophilia.
aminocaproic acid | tranexamic acid
39
any patient less than 2 w/ hemophilia and no prior bleeding events what do you need to do?
primary prophylaxis
40
once patients are over 2 w/ hemophilia and don't have bleeding episodes what do you?
primary prophylaxis
41
if a patient w/ hemophilia has a bleed what do you need to do?
secondary prophylaxis
42
what's the difference b/w primary and secondary prophylaxis
dosing of drugs
43
what virus is seen in recombinant factor VIII?
parovirus (comes from human cell line)
44
Does factor IX come from a human cell line?
No
45
do plasma derived factor or recombinant work better for hemophilia B?
plasma, but better to use recombinant factors because there is less risk
46
what are some ADRs w/ infusable factors
Chills, fever, HPOTN, edema thrombosis/ DVT anaphylaxis developing antibodies
47
if developing antibodies more common in hemophilia A or B?
hemophilia A
48
when should you suspect that the patient has produced IgG antibodies
post-infusion factor levels are lower than predicted
49
if you can't overcome the anitbodies produced by person, what must you give?
concentrated activated factors
50
what is prothrombin compelx concentrates (PCC)
plasma derived | contains Vit K dependent factors
51
what is used for bleeding episodes or prophylaxis before surgery. can't monitor effect w/ labs
Recombinant activated factor VII
52
what other meds do hemophiliacs need?
pain meds | intraarticular dexamethasone for joint pain
53
Acquired homeostatic disorder Due to underlying condition or illness Inappropriate systemic thrombosis followed by massive bleeding
Disseminated Intravascular Coagulation (DIC)
54
what are the three things that happen w/ DIC
hypercoagulability --> widespread inflammation thrombosis (consumes platelets and coags) bleeding
55
what are S/S of DIC?
``` Bleeding/oozing Thrombosis Petichiae/purpura Peripheral cyanosis Gangrene of distal extremities Hemorrhagic bullae ```
56
what labs w/ be abnormal w/ DIC (check serially)
``` 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
Tx for DIC (bleedign stage)
platelet infusion Fresh Frozen Plasma (FFP) Cryoprecipitate (can be used to replace fibrinogen)
58
patients with what dz can look like DIC
liver dz (but this has normal fibrinogen where DIC doesn't)
59
what drugs do you use for a patient in DIC in thrombotic phase?
heparin or LMWH
60
when can you consider antifibrinolytics w/ DIC
phase of fibrinolysis/ bleeding
61
what can you not co-administer w/ antifibrinolytics
heparin
62
what are the activated factors?
Prothrombin complex concentrates (PCC) | recombinant activated factor VII