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N935 Advanced Pathophysiology > hematologic - coagulation disorders > Flashcards

hematologic - coagulation disorders Flashcards

1
Q

hemophilia A concerns which factor

A

factor VIII (8)

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2
Q

hemophilia B concerns which factor

A

factor IX (9)

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3
Q

for surgery, individual clotting factor levels of ____ provide adequate hemostasis

A

20-25%

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4
Q

what products are available to treat single deficiencies?

A

factor concentrates, recombinant factors, FFP, gene therapy

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5
Q

how much FFP is needed to obtain a 20-30% increase in the level of any clotting factor?

A

15-20 mL/kg

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6
Q

hereditary deficiencies

A

hemophilia A, hemophilia B, von willebrand disease

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7
Q

acquired deficiencies

A

vitamin k deficiency (antibiotics, intestinal malabsorption, impaired nutrition), liver disease (parenchymal disease), disseminated intravascular coagulation (DIC), autoantibodies

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8
Q

hemophilia A is deficient in factor VIII that affects males or females?

A

males

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9
Q

severe hemophilia A

A

<1% factor VIII activity, needs factor VIII concentrate infusions at home

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10
Q

diagnosis of hemophilia A

A

prolonged aPTT, specific factor testing, and gene testing

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11
Q

anesthesia and hemophilia A

A

hematology consult, bring factor VIII level >50% prior to surgery, FFP, cryo, TXA
mild - DDAVP 30-90mins prior to surgery
moderate to severe- factor VIII concentrate

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12
Q

half life of Factor VIII

A

12 hours, but 6 hours in kids so may need redosing for days to weeks after surgery

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13
Q

hemophilia B affects males or females?

A

males

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14
Q

factor IX levels below ____ are associated with severe bleeding

A

<1%

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15
Q

mild hemophilia B

A

levels between 5-40%, isn’t detected until surgery or dental procedure

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16
Q

diagnosis of hemophilia B

A

prolonged aPTT, specific factor testing, and gene testing

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17
Q

anesthesia and hemophilia B

A

hematology consult
replacement therapy with recombinant factor IX, purified factor IX, prothrombin complex concentrate
TXA

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18
Q

prothrombin complex concentrate contains which factors

A

II, VII, IX, X

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19
Q

factor IX half life

A

18-24 hours

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20
Q

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

A

von willebrand disease

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21
Q

von willebrand disease is a family of disorders caused by

A

quantitative and/or qualitative defects

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22
Q

von willebrand factor mediates

A

platelet adhesion and prolongs factor VIII’s half life

23
Q

where is VWF synthesized and stored?

A

endothelial cells, platelets

24
Q

VWF action

A

platelet adhesion, platelet aggregation, carrier molecule for factor VIII and cofactor for factor IX

25
Q

type 1 von willebrand disease

A

most common (60-70% of patients)
mild-moderate reduction in level of VWF
mild bleeding symptoms

26
Q

type 2 von willebrand disease

A

9-30% of patients
qualitative defect of VWF
4 subtypes

27
Q

type 3 von willebrand disease

A

<1% of patients, nearly undetectable levels, severe quantitative phenotype

28
Q

platelet-pseudo type von willebrand disease

A

defect in the platelet’s GIb receptor

29
Q

treatment for type 1 von willebrand disease

A

desmopressin

30
Q

treatment for type 2 or 3 von willebrand disease

A

factor VIII concentrate, monitor factor levels, platelet transfusion

31
Q

replacement therapy goals in von willebrand disease in major surgery

A

maintain factor VIII level >50% for 1 week

prolonged treatment in type 3 patients >7 days

32
Q

replacement therapy goals in von willebrand disease in minor surgery

A

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

and then >20-30% for addt. 4-7 days

33
Q

replacement therapy goals in von willebrand disease in dental extraction

A

single infusion to achieve factor VIII levels >50%

desmopressin prior to procedure for type 1

34
Q

spontaneous or posttraumatic bleeding replacement therapy goal in von willebrand disease

A

single infusion of 20-40 units/kg

35
Q

factor VIII and IX deficiency can be treated with

A

FFP

36
Q

in disseminated intravascular coagulation (DIC) thrombin

A

is generated in response to an insulting factor leading to intravascular clotting that disseminates everywhere forming blood clots throughout the body and depleting coagulation factors and platelets, fibrinolysis is also activated and causes bleeding

37
Q

massive tissue destruction, sepsis, and endothelial injury causes

A

release of tissue factor

38
Q

release of tissue factor leads to

A

widespread microvascular thrombosis that leads to vascular occlusion and ischemic tissue damage

39
Q

activation of plasmin leads to

A

fibrinolysis and bleeding

40
Q

consumption of clotting factors and platelets leads to

A

bleeding

41
Q

symptoms of DIC

A

chest pain, SOB, leg pain, PE, DVT, ekg changes, problems speaking or moving

42
Q

in DIC hemorrhages occur simultaneously from ___ sites while there is ongoing thrombosis in ____

A

distant sites; the microcirculation

43
Q

causes of DIC

A

sepsis, surgery, trauma, cancer, pregnancy complications, snake bites (venom), frostbite, burns, transfusion reaction

44
Q

acute DIC

A

processes of coagulation and fibrinolysis are dysregulated

widespread clotting with resultant bleeding

45
Q

tissue factor binds with ___ and activates ___ to form ____

A

FVIIa; IX and Xl; thrombin and fibrin

46
Q

fibrinolysis creates

A

fibrin degradation products that inhibit platelet aggregation, antithrombin activity, and impair fibrin polymerization —> BLEEDING

47
Q

platelet consumption leads to

A

thrombocytopenia

48
Q

excess and unregulated thrombin generation causes

A

consumption of coagulation factors and increased fibrinolysis

49
Q

excess thrombin in DIC leads to

A

increased coagulation consumption, decreased anticoagulants, decreased antifibrinolysis, increased fibrinolysis, decreased platelets

50
Q

blood tests in DIC

A 
low platelets
low fibrinogen
high PT/INR
high PTT
high D-dimer
51
Q

thrombomodulin

A

binds to thrombin and decreases the proinflammatory response

52
Q

when would heparin be appropriate for DIC treatment

A

early or highly prothrombotic states

53
Q

considerations with heparin administration for DIC

A

high risk of additive bleeding
shown to reduce end organ dysfunction
DC’d once overt bleeding starts
difficult to monitor because PTT is already prolonged

54
Q

early hyperfibrinolysis may be treated with

A

transexamic acid (TXA)

N935 Advanced Pathophysiology (15 decks)

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