hemorrhagic disorders Flashcards

1
Q

two functions of vWF

A
  1. adhesion of platelets to endothelial cells

2. binds factor VIII in circulation and protects it

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2
Q

treatment for Von Willebrand Disease

A
  1. hormones- help with heavy menstrual bleeding
  2. desmosomes- help release vWF and FVIII from endothelial cells
  3. anti-fibrinolytics- helps stabilize clot on mucosal lining
  4. blood products containing clotting factors VIII and vWF
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3
Q

lab results of Von Willebrand Disease

A
  • normal PT, aPTT, and TT
  • low vWF antigen and activity and factor VIII level
  • PFA-100 level will be prolonged
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4
Q

clinical manifestations

A
  • menorrhagia
  • abnormal mucocutaneous bleeding
  • GI bleeding
  • epistaxis
  • heavy abnormal bleeding post surgery
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5
Q

what type of inheritance pattern is von willebrand disease?

A

autosomal co dominant

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6
Q

why is von willebrand disease difficult to diagnose?

A

factors like stress, anxiety, and menstrual cycle effect levels of vWF, ask about family history and history of abnormal bleeding

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7
Q

T/F people with von willebrand disease experience joint and muscle bleeding

A

false

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8
Q

______ is found in 75-95% of women with von willebrand disease

A

menorrhagia

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9
Q

average length of time from first bleeding experience to diagnosis is ____ years

A

17

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10
Q

what is the most common inherited bleeding disorder?

A

von willebrand disease

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11
Q

hemarthrosis, joint pain, edema, bleeding into muscle, GU bleeding, CNS bleeding is associated with

A

hemophilia A and B

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12
Q

hemophilia A is associated with what factor

A

8

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13
Q

hemophilia B is associated with what factor

A

9

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14
Q

what would a lab for a patient who has hemophilia show?

A

normal PT and TT
prolonged aPTT
normal PFA-100
normal vWF antigen and activity

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15
Q

< 1% of either factor 8 or 9 results in

A

spontaneous bleeding

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16
Q

adding factor 8 and 9 results in

A

cumulative affect where risk of bleeding decreases

17
Q

treatment for hemophilia

A
  • DDAVP for hemophilia A where it can raise VIII 3 fold before surgery
  • transfusions replace deficit
18
Q

what disease are people with hemophilia at risk due to the treatment?

A

HIV- hepatitis C

19
Q

orthopedic complication of hemophilic patients

A
  • recurrent joint bleeds
  • cartilage damage
  • accelerated arthritis
  • muscle wasting
  • need for joint replacement
20
Q

Hemophilia show what type of inheritance pattern ?

A

x-linked

21
Q

Hemophilia is x-lnked recessive… how would women have hemophilia?

A
  • two parents who are carriers
  • lyonization
  • spontaneous mutation
  • turners syndrome
22
Q

factor XI deficiency

A
  • autosomal recessive
  • Ashkenazi Jews
  • NO FACTOR CONCENTRATE
  • treat with plasma
  • post-surgical bleeding and mucocutaneous bleeding