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IBI Test 3 > B3-068 Big Case: Hemophilia > Flashcards

B3-068 Big Case: Hemophilia Flashcards

1
Q

quantitative types of vWD

A

1 and 3

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2
Q

qualitative types of vWD

A

type 2

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3
Q

platelet plug

A

primary hemostasis

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4
Q

fibrin meshwork stabilized by cross linking

A

secondary hemostasis

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5
Q

extrinsic pathway

A

tissue factor
factor VII

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6
Q

intrinsic pathway

A

prekallikrein
HMWK
XII
XI
IX
VIII

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7
Q

common pathway

A

X
V
II (prothrombin)
I (fibrinogen)

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8
Q

fibrin stabilization

A

XIII

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9
Q

PFA-100 screens for

A

primary hemostasis
-vWF
-platelets

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10
Q

PTT screen for

A

intrinsic pathway
-XII, XI, IX, VIII

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11
Q

PT screens for

A

extrinsic pathway
VII, tissue factor

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12
Q

D-dimer screens for

A

fibrinolysis

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13
Q

skin/mucosal bleeds indicates issue with

A

primary hemostasis

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14
Q

purpura, petechiae, ecchymosis indicate issue with

A

primary hemostasis

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15
Q

spontaneous bleeding indicates issue with

A

primary hemostasis

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16
Q

bleeds into soft tissue, muscle, joints indicate issue with

A

secondary hemostasis

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17
Q

hemarthrosis indicates issue with

A

secondary hemostasis

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18
Q

bleeding with trauma indicates issue with

A

secondary hemostasis

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19
Q

acquired factor deficiencies

secondary hemostasis

A

liver
DIC
vitamin K deficiency

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20
Q

factor inhibitors

secondary hemostasis

A

lupus anticoagulant
heparin therapy
specific factor inhibitors

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21
Q

factor VIII comes from the

A

liver

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22
Q

half life of unbound VIII

A

2.4 h

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23
Q

half life of factor VIII if bound to vWF

A

12 hrs

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24
Q

VIII is a cofactor for

A

factor IX

form Xase complex

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25
Q

VIII is a cofactor for

A

factor IX

form Xase complex

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26
Q

factor VIII can be measured via

A

PTT
FVIII clotting assay

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27
Q

vWF comes from

A

endothelial cells and megakaryocytes

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28
Q

vWF can be measured via

A

PFA-100 (screen)
vWF antigen (quantify)
vWF activity (quality)

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29
Q

hemophilla A is caused by decreased

A

factor VIII

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30
Q

hemophillia B is caused by decreased

A

factor IX

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31
Q

hemophilia A is [more/less] common than B

A

more

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32
Q

Hemophilia A and B inheritance pattern

A

X linked recessive

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33
Q

30% of hemophilia A and B are caused by

A

new mutations, no family hx

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34
Q

spontaneous or traumatic bleeding into joints, soft tissue, or muscle

A

hemophilia A/B

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35
Q

hemophilia A and B can cause life threatening bleeding into

A

CNS, oropharyngeal space, retroperitoneal space

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36
Q

self-limited hematuria

A

hemophilia A/B

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37
Q

contractures, joint deformaties secondary to hemarthrosis

A

hemophilia A/B

38
Q

hemophilia can be diagnosed via

A

prolonged PTT
decreased factor VIII or IX level

extent of prolongation does not correlated with factor level

39
Q

only way to distinguish hemophilia A from B

A

decreased factor VIII or IX level

40
Q

disease severity of hemophilia is associated with

A

residual factor level

41
Q

severe hemophilia

A

<1%

risk of spontaneous bleeding

42
Q

moderate hemophilia

A

1-5%

43
Q

mild hemophilia

A

6-30%

usually only have bleeds in relation to trauma

44
Q

what products should never be used to treat hemophilia

A

cryoprecipitate

45
Q

leading causes of death in hemophilia patients prior to 1980s

A

HIV and HCV

due to transfusion with contaminated blood products

exceedingly rare now

46
Q

treatment for hemophilia

A

factor VIII or IX concentrates
supportive: DDAVP, antifibrinolytics

DDAVP increases vWF, severe will not respond to this

DDAVP=desmopressin

47
Q

Dose calculation for factor VIII concentrate

A

(target FVIII level- baseline FVIII level) x body weight (kg) x 0.5 (unit/kg)

48
Q

dosing for factor VIII concentrate

A

twice daily or continuous

49
Q

dose calculation for factor IX concentrate

A

(target FIX level- baseline FIX level) x body weight (kg) x 1 (unit/kg)

50
Q

dosing for factor IX concentrate

A

once daily or continuous

51
Q

target factor levels for primary prophylaxis

A

1%

52
Q

target factor level: surgical prophylaxis

A

100% for 7-10 days, then tapered based on risk of bleeding

53
Q

target factor level: mild bleeding

A

30-50% for several days

54
Q

target factor level: severe bleeding

A

100% for several days

55
Q

incidence of antibodies to FVIII

A

5-10% of all cases
20% of severe cases

56
Q

incidence of antibodies to FIX

A

3-5% of all cases

57
Q

risk factors for FVIII or FIX inhibitors

A

severe deficiency
family hx
african descent
large gene deletions or rearrangements
intensive therapy

58
Q

hemophilia due to inhibitors shows a

A

lack of response to therapeutic dose of factor concentrates

59
Q

hemophilia due to factor inhibitors will develop ….. in life

A

early

60
Q

laboratory testing of hemophilia inhibitors

A

PTT 1:1 mixing study
bethesda assay

61
Q

<5 BU

A

low responder

62
Q

.>10 BU

A

high responder

more difficult to treat, use bypass agents like FVIIa to activate X

63
Q

1 BU

A

amount of inhibitor that neutralized 50% factor activity at 2 hr

64
Q

management of acute bleeding in low responders

A

high dose recombinant human or porcine factor concetrate

65
Q

management of acute bleeding in high responder

A

bypass agents (prothrombin complex concentrates) or recombinant factor VIIa

bypasses need for FVIII and FIX, but extremely difficult to titrate

66
Q

treatments for inhibitor reduction in hemophilia

A

immune tolerance induction
immunosuppression, anti CD20 (rituximab)

not super effective

67
Q

FXII, HMWK, prekallikrein deficiencies cause

A

prolonged PTT but no bleeding tendency

68
Q

FXI deficiency causes

A

variable bleeding tendency

depends on severity of mutation

69
Q

combined FV and FVIII deficiency is caused by

A

lack or dysfunction of chaperone protein

70
Q

hemophilia A is due to deficiency in factor

A

8

71
Q

in hemophilia A, deficiency of factor 8 risrupts the

A

intrinsic and common pathways

causes decreased factor X

72
Q

hemarthroses

A

hemophilia A

73
Q

dental procedures could cause hemorrhage

A

hemophilia A

74
Q

hemophilia A leads to prolonged [PT/PTT]

A

PTT

75
Q

desomopressin can be used to treat

A

hemophilia A

induces platelets and endothelial cells to release factor 8

76
Q

inheritance pattern: hemophilia

A

X linked recessive

77
Q

hemophilia B is due to deficiency of

A

factor IX

78
Q

SLE can be associated with

A

hemophilia B

79
Q

factor deficiencies that show no bleeding tendencies

A

Factor XII
HMWK
prekallikrein

80
Q

inherited clotting disorders are generally

A

uncommon

81
Q

deficiency of common factors is more likely to impact [PTT/PT]

A

PTT

82
Q

when treating factor deficiencies, always opt for …… as they are not infectious

A

concentrated producted

blood products are more commonly infectious

83
Q

Factor VIII catalyes the reaction of

A

FX to FXa

84
Q

screening test for VIII deficiency

A

PTT

85
Q

definitive test for FVIII deficiency

A

FVIII clotting assay

normal 50-150%

86
Q

screening test for vWF deficiency

A

PFA-100

87
Q

in type 2 vWD, the patient will have low levels of

A

VIII

2.4 hour half life without vWF

88
Q

how would you distinguish vWD normandy (type 2) from FVIII deficiency?

A

vWF normandy is autosomally inherited
FVIII is X linked recessive

89
Q

hemophilia is symptomatic at ….% functionality

A

30

90
Q

should hemphilia be excluded from the differential without family hx?

A

no; 30% of hemophilia cases are caused by de novo mutations

IBI Test 3 (20 decks)

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