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HEM Exam 1 > Hemorrhagic Disorders > Flashcards

Hemorrhagic Disorders Flashcards

1
Q

two functions of VWF

A
  • helps platelets stick to injured subendothelium

- carries and protects factor 8 around circulation

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2
Q

most common inherited bleeding disorder is

A
  • von Willebrand disease
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3
Q

inheritance of vWD

A
  • autosomal codominant
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4
Q

vWD caused by

A
  • deficiency or synthesis of dysfunctional form of vWF
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5
Q

Type 1 vWD

A
  • quantitative disorder

- 30% normal levels

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6
Q

VWD clinical manifestations

A
  • mucocutaneous bleeding

- excessive bleeding after surgery

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7
Q

VWD excessive bleeding after surgery where

A
  • mucosal surfaces
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8
Q

VWD mucocutaneous bleeding where

A
  • heavy menstrual
  • easy bruising
  • GI bleeding
  • nosebleeds
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9
Q

VWD excessive bleeding after which surgeries

A
  • tooth extraction
  • tonsillectomy
  • hysterectomy
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10
Q

VWD joint and muscle bleeding?

A
  • none!
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11
Q

most common symptom in women with VWD

percent affected

A
  • menorrhagia (heavy mentrual bleeding)

- 75-95%

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12
Q

after length of time from first bleeding episode to diagnosis of VWD in women

A
  • 17 years
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13
Q

VWD levels increased in

A
  • pregnancy

- estrogen in OCP and HRT

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14
Q

effect of Type O blood on VWF

A
  • makes VWF low
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15
Q

diagnosing VWD

A
  • personal history of abnormal bleeding
  • family history of abnormal bleeding
  • low levels of VWF
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16
Q

how low does VWF need to be for VWD

A
  • < 30%
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17
Q

treatment of VWD

A
  • hormone manipulations
  • desmopressin
  • antifibrinolytics
  • blood products and clotting factors
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18
Q

desmopressin MOA for treatment of VWD

A
  • releases factor 8 and VWF from endothelial stores
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19
Q

desmopressin effects with prolonged use in VWD

A
  • hyponatremia
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20
Q

antifibrinolytics MOA in VWD

A
  • stabilize clot on mucosal surfaces
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21
Q

blood products and clotting factors in VWD treatment contain

A
  • Factor 8 and VWF
22
Q

hemophilia A is deficient in

A
  • factor 8
23
Q

hemophilia B is deficient in

A
  • factor 9
24
Q

hemophilia A and B inheritance

A
  • X linked
25
Q

hemophilia A and B found mostly in

A
  • men
26
Q

PT of hemophilia A

A
  • normal
27
Q

PT of type 1 VWD

A
  • normal
28
Q

aPTT of hemophilia A

A
  • prolonged
29
Q

aPTT of type 1 VWD

A
  • normal

- slightly prolonged

30
Q

TCT of hemophilia A

A
  • normal
31
Q

TCT of type 1 VWD

A
  • normal
32
Q

PFA-100 of hemophilia A

A
  • normal
33
Q

PFA-100 of type 1 VWD

A
  • prolonged
34
Q

factor 8 level of hemophilia A

A
  • low
35
Q

factor 8 level of type 1 VWD

A
  • low

- not very low

36
Q

VWF antigen hemophilia A

A
  • normal
37
Q

VWF antigen type 1 VWD

A
  • low
38
Q

VWF activity hemophilia A

A
  • normal
39
Q

VWF activity type 1 VWD

A
  • low
40
Q

spontaneous bleeding presents with a factor 8 or 9 level of

A
  • <1%
41
Q

hemophilia bleeding sites

A
  • hemarthorsis
  • bleeding into muscles
  • CNS bleeding
  • GU bleeding
  • retroperitoneal bleeding
  • oro- and nasopharynx
42
Q

orthopedic complications of hemophilia

A
  • recurrent joint bleeds
  • synovitis
  • cartilage damage
  • accelerated arthritis
  • need for joint replacement
43
Q

treatment of hemophilia

A
  • desmopressin

- replace deficient factor

44
Q

what form of hemophilia do you use desmopressin for

A
  • mild hemophilia A
45
Q

hemophilia inhibitors

A
  • patients with hemophilia that develop neutralizing antibodies to clotting factor concentrates
46
Q

hemophilia inhibitors common in which form ion hemophilia

A
  • A
47
Q

women can have low levels of factor 8 or 9 due to a process called

A
  • lyonization
48
Q

what is lyonization

A
  • one of the copies of X chromosome in females is inactivated
49
Q

inheritance of factor 9 deficiency

A
  • autosomal recessive
50
Q

factor 9 deficiency most commonly found in which population

A
  • Ashkenazi jews

HEM Exam 1 (22 decks)

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