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Pathophys II > Coagulation Disorders > Flashcards

Coagulation Disorders Flashcards

1
Q

Hereditary deficiencies include

A

Hemophilia A
Hemophilia B
Von Willebrand disease

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2
Q

Acquired coagulation deficiencies include

A

vitamin K deficiency- antibiotics, intestinal malabsorption, impaired nutrition
liver disease- parenchymal disease
disseminated intravascular coagulation
autoantibodies

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3
Q

Treatment of a single-factor deficiency depends on

A

the severity of the deficiency

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4
Q

For surgical purposes, individual clotting factor levels of

A

20-25% provide adequate hemostasis

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5
Q

List the products available to treat single deficiencies.

A

factor concentrates
recombinant factors
gene therapy
FFP- 15-20 mL/kg of FFP is needed to obtain a 20% to 30% increase in the level of any clotting factor

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6
Q

Duration of effect for replacement therapy depends on

A

the turnover time of each factor

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7
Q

Hemophilia A is a result of

A

Factor VIII gene deficiency
very large gene on the X chromosome
affects 1 in 5,000 males- can be inherited or gene mutation

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8
Q

Severe hemophilia is the

A

inversion or deletion of major portions of the Factor VIII gene

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9
Q

Clinical severity of hemophilia A is best correlated with the

A

factor VIII activity level

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10
Q

Describe severe hemophilia A.

A

<1% factor VIII activity

  • diagnosed in childhood- spontaneous hemorrhage into joints, muscles, and vital organs
  • requires factor VIII concentrates
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11
Q

Describe mild hemophilia A.

A

6-30% factor VIII activity- may go undiagnosed until adulthood & they undergo major surgery

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12
Q

Diagnosis of hemophilia A is through

A

prolonged aPTT, specific factor testing, and gene testing

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13
Q

Anesthetic considerations for the patient with Hemophilia A include

A

HEMATOLOGY CONSULT
factor VIII level should be brought to at least >50% prior to surgery
mild hemophilia A- DDAVP 30-90 minutes prior to surgery
Moderate to severe hemophilia A- Factor VIII concentrate
FFP & cryo
Consider TXA as an adjunct

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14
Q

Factor VIII concentrate half-life is

A

approximately 12 hours in adults (short as 6 hours in children)
may require replacement therapy for days to weeks after surgery

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15
Q

Describe hemophilia B

A

similar clinical picture as hemophilia A

-1:30,000 males (also X-linked & less common)

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16
Q

How is hemophilia B diagnosed?

A

prolonged aPTT, specific factor testing, and gene testing

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17
Q

Describe mild vs. severe hemophilia B.

A

Factor IX levels below 1% are associated with severe bleeding
-mild disease (levels between 5-40%) often not detected until surgery or dental procedure

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18
Q

Describe the anesthesia implications for hemophilia B

A 
similar to hemophilia A
HEMATOLOGY consult
Replacement therapy- recombinant F-IX, purified F-IX, prothrombin complex concentrate contain II, VII, IX, and X, increased risk of thrombotic events- especially in orthopedic surgery 
-continue replacement therapy
Consider tranexamic acid as adjunct
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19
Q

Describe the different anesthesia implications for hemophilia A versus hemophilia B

A

With continuance of replacement therapy, factor IX half-life is 18-24 hours so not dosed as frequently
absorbed into collagen & vasculature

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20
Q

The most common congenital bleeding disorder in the world is

A

Von Willebrand’s disease

- more prevalent in person of European descent

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21
Q

Von Willebrand disease is

A

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

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22
Q

Von Willebrand factor mediates

A

platelet adhesion and prolong factor VIII’s half-life

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23
Q

Von Willebrand is synthesized and stored in

A

endothelial cells & platelets

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24
Q

Describe the action of Von Willebrand.

A

dual role in hemostasis affecting both platelet function & coagulation

  1. platelet adhesion- vascular site of injury to PLT’s GIb receptor
  2. platelet aggregation- PLT GIIb/IIIa receptor to PLT GIIB/IIIa receptor
  3. Carrier molecule for factor VIII and cofactor for factor IX
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25
Q

Describe type 1 of Von Willebrand disease

A

most common 60-70% of patients-
mild-moderate REDUCTION in level of vWF
mild bleeding symptoms- easing bruising, nosebleeds

26
Q

Describe type 2 of Von Willebrand disease

A

9-30% of patients
QUALITATIVE DEFECT OF VWF
4 subtypes

27
Q

Describe type 3 of Von Willebrand disease

A

<1% of patients

nearly undetectable, severe quantitative phenotype

28
Q

Describe platelet pseudo type Von WIllebrand disease

A

defect in the platelet’s GIb receptor

29
Q

What determines the approach to treatment in Von Willebrand’s disease?

A

the specific type of von Willebrand disease & the location and severity of bleeding

30
Q

Describe the replacement therapy goals in von Willebrand disease.

A

Major surgery- maintain factor VIII level >50% for 1 week
minor surgery- maintain factor VIII level >50% for 1-3 days
Dental extraction- single infusion to achieve factor VIII level >50%, desmopressin prior to procedure for type I
spontaneous or posttraumatic bleeding- usually single infusion of 20-40 units/kg

31
Q

Describe the difference in treatment for factor VIII & IX deficiency

A

FFP, factor concentrates, & cryo
FFP PCC, factor concentrates
cryo only works for factor VIII

32
Q

In normal hemostasis, thrombin stays at

A

the site of vascular injury

33
Q

In DIC, thrombin is

A

generated in response to an insulting factor

34
Q

Insulting factors for DIC can be

A

endotoxins in sepsis or amniotic fluid embolism

35
Q

In DIC, the generation of thrombin leads to

A

intravascular clotting which then disseminates through the body causing end-organ dysfunction
coagulation factors depleted ad platelets are used up or become dysfunctional
fibrinolysis is also activated and results in bleeding

36
Q

Describe the symptoms of DIC

A

related to widespread clot formation
-chest pain, SOB, leg pain, problems speaking or moving
patients may present with either clotting or bleeding or both

37
Q

With DIC hemorrhages occur simultaneously from distant sites

A

where there is ongoing thrombosis in the microcirculation

-IV sites, catheters, & drains

38
Q

Causes of DIC include

A 
sepsis- bacterial, viral, fungal
surgery
trauma
cancer- more chronic, insidious onset, often leukemias
pregnancy complications- amniotic fluid embolism, HELLP syndrome
snake bites- venom
frostbite
burns 
transfusion reaction
39
Q

acute DIC is when the processes of

A

coagulation & fibrinolysis are dysregulated

40
Q

Acute DIC causes

A

widespread clotting with resultant bleeding

  1. fibrin deposits as thrombosis in the circulation
  2. depletion of platelets & clotting factors
41
Q

Regardless of the triggering event of DIC, once initiated, the pathophysiology of DIC

A

is similar in all conditions

42
Q

With acute DIC, tissue factor

A

may be present in many cell surfaces (endothelial, macrophages, monocytes) and tissues (lung, brain, placenta)
tissue factor is exposed & released
binds with FVIIa and activates IX and X to form thrombin and fibrin in the common final pathway

43
Q

Fibrinolysis creates fibrin degradation products that

A

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

44
Q

The paradoxical effect is when

A

coagulation inhibitors are also consumed
decreased inhibitor levels will permit more clotting
increased clotting leads to more clotting
thrombocytopenia occurs due to platelet consumption
dysfunctional platelets occur due to inflammatory processes

45
Q

Blood tests in DIC include

A

DIC panel- these labs in isolation are not helpful
-low platelets (93% of cases)- but also platelet dysfunction
-low fibrinogen
- high INR & PT
-high PTT
-high D-dimer (FDP) (Variable)
TEGs & ROTEMs

46
Q

Excess thrombin in DIC results in

A

lots and lots of clotting

  • -> depletion of platelets and clotting factors
  • -> fibrin degradation products (FDP)
47
Q

Excess and unregulated thrombin generation causes

A

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

48
Q

Consumption of anticoagulant proteins with high antifibrinolytic activity and platelet aggregation also induced by thrombin can lead to

A

thrombotic complications

49
Q

Summarize the role of excess thrombin in DIC.

A

increased coagulation consumption–> bleeding
increased fibrinolysis
decreased platelets–> platelet dysfunction & platelet aggregation
decreased anticoagulant leading to thrombosis
decreased antifibrinolysis

50
Q

Treatment of DIC includes:

A

recognition
treat the underlying condition- infection give abx or trauma resus or removal of insult
& supportive

51
Q

Describe the supportive treatments of DIC

A 
platelets
cryo
fibrinogen concentrate
FFP
heparin, TX
PCCs (contain fewer natural anticoagulants than FFP)
52
Q

Throbomodulin binds to

A

thrombin and decreases the proinflammatory response

limited clinical trials

53
Q

Thrombin is a

A

potent proinflammatory protein & platelet aggregator
neutralizing thrombin effect is crucial
can give throbomodulin
direct thrombin inhibitors- no RCTs yet

54
Q

The use of heparin in DIC is

A

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

55
Q

In severe trauma, excessive _____ is generated to rescue the host from excessive bleeding

A

thrombin

56
Q

Early hyperfibrinolysis may be treated with

A

tranexamic acid

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

57
Q

In a severe trauma while thrombin is being generated, ______ is also generated

A

plasmin which breaks down the microvascular clot

unfortunately it is not able to isolate leading to widespread hemorrhage

58
Q

Blood product support for DIC includes

A

platelets–> cryo or fibrinogen concentrate–>FFP

59
Q

In presenting procoagulant DIC- consider

A

anticoagulation first & blood product support for bleeding or invasive procedures
-LMWH (thrombomodulin trial stages only)

60
Q

In presenting hyperfibrinolysis over procoagulant process is anticipated provide

A

TXA along with blood product support

once bleeding has been controlled, consider anticoagulant treatment to control the ongoing thrombin generation

61
Q

A thorough history for patients with clotting disorders should include

A

excessive bleeding after dental extraction?, medications such as ASA, NSAIDs, or gingko, family history of inherited bleeding disorders

62
Q

Some healthy individuals may have

A

abnormal lab values and patients with mild hemophilia or vWD may not display abnormal values

Pathophys II (43 decks)

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