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N935 Advanced Pathophysiology > Week 3: Coagulation Disorders > Flashcards

Week 3: Coagulation Disorders Flashcards

1
Q

What does the treatment of single-factor deficiency depend on?

A

the severity of the deficiency

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2
Q

For surgery what are the adequate individual clotting factor levels for hemostasis?

A

20-25%

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3
Q

What products are available to treat single factor deficiencies? (4)

A

factor concentrates
recombinants factors
FFP
gene therapy

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4
Q

What does the duration of the effect for replacement therapy depend on?

A

the turnover time of each factor

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5
Q

What are the three hereditary deficiencies?

A

hemophilia A
hemophilia B
Von Willebrand disease

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6
Q

What are the four acquired deficiencies?

A

vitamin K deficiency
liver disease
disseminated intravascular coagulation
autoantibodies

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7
Q

What factor is deficient in hemophilia A?

A

factor VIII

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8
Q

What is factor VIII?

A

gene is a very large gene on the X chromosome

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9
Q

Who does Hemophilia A affect?

A

1:5000 males

can be inherted or gene mutation

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10
Q

How is the clinical severity of hemophilia A determined?

A

best correlated with factor VIII activity level

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11
Q

What is severe hemophilia A?

A

<1% factor VIII activity

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12
Q

What is mild hemophilia A?

A

6-30% factor VIII activity

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13
Q

How do you diagnosis hemophilia A?

A

prolonged PTT, specific factor testing, and gene testing

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14
Q

For surgery, hemophilia A patients require

A

hematology consult

>50% factor VIII level

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15
Q

How do you treat mild hemophilia A prior to surgery?

A

DDAVP 30-90 minutes prior to surgery

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16
Q

How do treat moderate to severe hemophilia A prior to surgery?

A

factor VIII concentrate
may require replacement therapy for days to weeks after surgery
FFP and cryo
TXA as adjunct

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17
Q

What is the half life of Factor VIII in adults? pediatrics?

A

12 hours in adults

6 hours in peds

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18
Q

Hemophilia B is what factor deficiency?

A

Factor IX

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19
Q

Who does hemophilia B affect?

A

1:30,000 males (Xlinked and much less common)

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20
Q

What factor level for hemophilia B is associated with severe bleeding?

A

1%

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21
Q

What % factor levels of hemophilia B is mild disease?

A

levels between 5-40%

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22
Q

How do you diagnosis Hemophilia B?

A

prolonged aPTT, specific factor testing, and gene testing

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23
Q

WHo should be consulted before hemophilia B patients to go to surgery?

A

hematology consult

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24
Q

What is the replacement therapy for hemophilia B patients?

A

recombinant F IX
purified F-IX
prothrombin concentrate (PCCs) contain 2,9,10

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25
Q

What is the half life of factor IX?

A

18-24 hours

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26
Q

What is the most common congenital bleeding disorder in the world?

A

von willebrand disease

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27
Q

What is von willebrand disease?

A

a family of disorders caused by quantitative and/or qualitative defect

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28
Q

What does von willebrand factor do?

A

mediates platelets adhesion and prolongs factor VIII’s half life

29
Q

Where is von willebrand factor stored and synthesized?

A

endothelial cells and platelets

30
Q

What is the overall role of von willebrand factor?

A

dual role in hemostasis, affecting both platelet function and coagulation

31
Q

What are the 3 specific roles of VWF?

A

platelet adhesion- vascular site of injury to PLT’s GIb receptor
Platelet aggregation- PLT GIIb/IIIa receptor to PLT GIIB/ IIIa receptor
carrier molecule for factor VIII and cofactor for factor IX

32
Q

What is type 1 VWD?

A

mild-moderate reduction in level of vWF

mild bleeding symptoms- easy bruising, nosebleeds

33
Q

What is the most common type of VWD?

A

type 1

34
Q

What is type 2 VWD?

A

qualitative defects of vWF

4 subtypes

35
Q

what is the % of type 2 vWD?

A

9-30% of patients

36
Q

What is type 3 vWD?

A

nearly undetectable, severe quantitative phenotype

37
Q

What is platelet-pseudo type wvd?

A

defect in platelet’s GIb receptor

38
Q

what determines the treatment of vWD?

A

location and severity of bleeding

39
Q

What are the replacement goals of major surgery with vWD patient?

A

maintain factor VIII levels >50% for 1 week

prolonged treatment in type 3 patients >7days

40
Q

What are the replacements goals of minor surgery with vWD patient?

A

maintain factor VIII level >50% for 1-3 days

maintain factor VIII level >20%-30% for an additional 4-7 days

41
Q

How would you treat a vWD patient for a dental extraction?

A

single infusion to achieve factor VIII level <50%

desmopressin prior to procedure for type 1

42
Q

What is generated in response to an insulting factor during DIC?

A

thrombin

43
Q

What is an insulting factor?

A

endotoxins in sepsis, amniotic fluid embolism

44
Q

What does thrombin cause?

A

intravascular clotting then dissemination

45
Q

What do the blood clots cause in DIC?

A

end-organ dysfunction

46
Q

What is depleted or used up in DIC?

A

coagulation factors depleted and platelets are used up

47
Q

What is activated in. DIC and results in bleeding?

A

fibrinolysis

48
Q

what are the symptoms of DIC?

A

related to widespread clot formation
chest pain, shortness of breath, leg pain, problems with speaking or moving
patients can be clotting or bleeding or both

49
Q

Where do hemorrhages occur during DIC?

A

they occur simultaneously from distant sites while there is ongoing thrombosis in the microcirulation
IV sites, catheters, drains

50
Q

What are the 9 causes of DIC?

A 
sepsis (bacterial, viral, fungal)
surgery
trauma
cancer (more chronic)
pregnancy complications (amniotic fluid embolism, HELLP syndrome)
snake bites (venom)
frostbite 
burns
transfusion reaction
51
Q

Acute DIC is

A

processes of coagulation and fibrinolysis are dysregulated

widespread clotting with resultant bleeding

52
Q

what are the two processes that cause bleeding and clotting?

A
  1. fibrin deposits as thrombosis in the circulation

2. depletion of platelets and clotting factors

53
Q

What is tissue factor?

A

present in many cell surfaces (endothelial macrophages, monocytes) and tissues (lung, brain, placenta)

54
Q

What happens with tissue factor in DIC?

A

exposed and released

binds wit FVIIa and activates IX and X to form thrombin and fibrin in the common final pathway

55
Q

Fibrinolysis creates a fibrin degradation products that cause what 3 things?

A

inhibit platelet aggregation
have antithrombin activity
impair fibrin polymerization
all contribute to bleeding

56
Q

What is the paradoxical effect of acute DIC?

A

coagulation inhibitors are also consumed= more clotting
increased clotting leads to more clotting
thrombocytopenia occurs due to platelet consumption
dysfunctional platelets occur due to inflammatory processes

57
Q

Thrombin allows for

A

depletion of platelets and clotting factors

fibrin degradation products

58
Q

What does the excess and unregulated thrombin generation causes

A

consumption of coagulation factors and increased fibrinolysis which in conjunction with platelet dysfunction can lead to bleeding

59
Q

What causes thrombotic complications?

A

consumption of anticoagulant proteins with high antifibrinolytic activity and platelet aggregation also induced by thrombin leads to complications

60
Q

What labs diagnosis DIC?

A 
DIC panel
low platelets
low fibrinogen
high INR and PT
high PTT
HIgh D dimer 
TEGs and ROTEMs
61
Q

How do you treat DIC?

A 
recongition
treat underlying conditions
infection- give antibotics
trauma- resuscitation, remove insult
supportive- give platelets, cryo, fibrinogen concentration
FFP
heparin TXA (if in coagulation phase)
PCCs (contain fewer natural anticoagulant than FFPs)
62
Q

Thrombin is

A

a potent proinflammatory protein and platelet aggregator

63
Q

Neutralizing what is crucial in acute DIC?

A

thrombin

64
Q

Throbomodulin

A

binds to thrombin and decreases the pro-inflammatory response (limited clinical trials)

65
Q

Direct thrombin inhibitors

A

no RCTs yet

66
Q

Heparin in DIC

A

variable outcomes
reserved for early or highly prothrombotic states
high risk of additive bleeding
reduce end-organ dysfunction
discontinued if/when overt bleeding starts
difficult to monitor because PTT is already prolonged due to coagulation factor consumption

67
Q

What is generated during severe trauma?

A

excessive thrombin to rescue the host from excessive bleeding
plasmin is also generated to breakdown the microvascular clots, but unfortunately is not able to isolate leading to widespread hemorrhage

68
Q

What can tranexamic acid treat?

A

early hyperfibrinolysis

consider single upfront bolus during period as later the balance is tipped toward thrombosis

N935 Advanced Pathophysiology (9 decks)

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