hematologic - coagulation disorders

Question 1

hemophilia A concerns which factor

Answer 1

factor VIII (8)

Question 2

hemophilia B concerns which factor

Answer 2

factor IX (9)

Question 3

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

Answer 3

20-25%

Question 4

what products are available to treat single deficiencies?

Answer 4

factor concentrates, recombinant factors, FFP, gene therapy

Question 5

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

Answer 5

15-20 mL/kg

Question 6

hereditary deficiencies

Answer 6

hemophilia A, hemophilia B, von willebrand disease

Question 7

acquired deficiencies

Answer 7

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

Question 8

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

Answer 8

males

Question 9

severe hemophilia A

Answer 9

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

Question 10

diagnosis of hemophilia A

Answer 10

prolonged aPTT, specific factor testing, and gene testing

Question 11

anesthesia and hemophilia A

Answer 11

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

Question 12

half life of Factor VIII

Answer 12

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

Question 13

hemophilia B affects males or females?

Answer 13

males

Question 14

factor IX levels below ____ are associated with severe bleeding

Answer 14

<1%

Question 15

mild hemophilia B

Answer 15

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

Question 16

diagnosis of hemophilia B

Answer 16

prolonged aPTT, specific factor testing, and gene testing

Question 17

anesthesia and hemophilia B

Answer 17

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

Question 18

prothrombin complex concentrate contains which factors

Answer 18

II, VII, IX, X

Question 19

factor IX half life

Answer 19

18-24 hours

Question 20

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

Answer 20

von willebrand disease

Question 21

von willebrand disease is a family of disorders caused by

Answer 21

quantitative and/or qualitative defects

Question 22

von willebrand factor mediates

Answer 22

platelet adhesion and prolongs factor VIII’s half life

Question 23

where is VWF synthesized and stored?

Answer 23

endothelial cells, platelets

Question 24

VWF action

Answer 24

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

Question 25

type 1 von willebrand disease

Answer 25

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

Question 26

type 2 von willebrand disease

Answer 26

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

Question 27

type 3 von willebrand disease

Answer 27

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

Question 28

platelet-pseudo type von willebrand disease

Answer 28

defect in the platelet's GIb receptor

Question 29

treatment for type 1 von willebrand disease

Answer 29

desmopressin

Question 30

treatment for type 2 or 3 von willebrand disease

Answer 30

factor VIII concentrate, monitor factor levels, platelet transfusion

Question 31

replacement therapy goals in von willebrand disease in major surgery

Answer 31

maintain factor VIII level >50% for 1 week | prolonged treatment in type 3 patients >7 days

Question 32

replacement therapy goals in von willebrand disease in minor surgery

Answer 32

maintain factor VIII level >50% for 1-3 days | and then >20-30% for addt. 4-7 days

Question 33

replacement therapy goals in von willebrand disease in dental extraction

Answer 33

single infusion to achieve factor VIII levels >50% | desmopressin prior to procedure for type 1

Question 34

spontaneous or posttraumatic bleeding replacement therapy goal in von willebrand disease

Answer 34

single infusion of 20-40 units/kg

Question 35

factor VIII and IX deficiency can be treated with

Answer 35

FFP

Question 36

in disseminated intravascular coagulation (DIC) thrombin

Answer 36

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

Question 37

massive tissue destruction, sepsis, and endothelial injury causes

Answer 37

release of tissue factor

Question 38

release of tissue factor leads to

Answer 38

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

Question 39

activation of plasmin leads to

Answer 39

fibrinolysis and bleeding

Question 40

consumption of clotting factors and platelets leads to

Answer 40

bleeding

Question 41

symptoms of DIC

Answer 41

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

Question 42

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

Answer 42

distant sites; the microcirculation

Question 43

causes of DIC

Answer 43

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

Question 44

acute DIC

Answer 44

processes of coagulation and fibrinolysis are dysregulated | widespread clotting with resultant bleeding

Question 45

tissue factor binds with ___ and activates ___ to form ____

Answer 45

FVIIa; IX and Xl; thrombin and fibrin

Question 46

fibrinolysis creates

Answer 46

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

Question 47

platelet consumption leads to

Answer 47

thrombocytopenia

Question 48

excess and unregulated thrombin generation causes

Answer 48

consumption of coagulation factors and increased fibrinolysis

Question 49

excess thrombin in DIC leads to

Answer 49

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

Question 50

blood tests in DIC

Answer 50

``` low platelets low fibrinogen high PT/INR high PTT high D-dimer ```

Question 51

thrombomodulin

Answer 51

binds to thrombin and decreases the proinflammatory response

Question 52

when would heparin be appropriate for DIC treatment

Answer 52

early or highly prothrombotic states

Question 53

considerations with heparin administration for DIC

Answer 53

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

Question 54

early hyperfibrinolysis may be treated with

Answer 54

transexamic acid (TXA)