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Liz's Hemostasis/Coagulation > Inherited Diseases of Coagulation > Flashcards

Inherited Diseases of Coagulation Flashcards

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1
Q

Importance of evaluating bleeding episodes in a patient’s history when a bleeding disorder is suspected

A

Is the bleeding out of proportion to the challenge/injury?

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2
Q

Importance of a physical examination in a patient’s history when a bleeding disorder is suspected

A

?

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3
Q

Importance of evaluating family history in a patient’s history when a bleeding disorder is suspected

A

Sex-linked vs. autosomal

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4
Q

Importance of evaluating drug history in a patient’s history when a bleeding disorder is suspected

A

?

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5
Q

Importance of evaluating the presence of existing diseases in a patient’s history when a bleeding disorder is suspected

A

?

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6
Q

Six types of coagulation disorders which may have normal screening tests

A
  • von Willebrand disease (many variations)
  • Mild hereditary factor deficiencies
  • Factor XIII deficiency
  • Dysfibrinogenemia
  • Mild qualitative platelet disorders
  • Mild acquired inhibitors
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7
Q

Factor VIII deficiency (Hemophilia A)

- How is it inherited?

A

Sex-linked recessive

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8
Q

Factor VIII deficiency (Hemophilia A)

- Treatment

A
  • Factor VIII concentrate

- DDAVP (vasopressin than stimulates teh production of VIII:C and VIII:vWF)

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9
Q

Factor IX deficiency (Hemophilia B)

- How is it inherited?

A

Sex-linked recessive

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10
Q

Factor IX deficiency (Hemophilia B)

- Treatment

A
  • Factor IX concentrates
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11
Q

von Willebrand disease

- How is it inherited?

A
  • Autosomal dominant

- Autosomal recessive

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12
Q

von Willebrand disease

- Bleeding episodes

A
  • Mucus membrane bleeding
  • Superficial wound bleeding
  • Bleeding w/ other hemostatic chanllenges (surgery, childbirth)
  • Menorrhagia
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13
Q

von Willebrand disease

- Coagulation test results

A
  • PLT: normal
  • BT: prolonged
  • PLT aggregation studies: absent w/ ristocetin, normal w/ other agonists
  • PT: normal
  • APTT: prolonged (usually)
  • VIII assay: decreased (usually)
  • VIII: vWF Ag decreased
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14
Q

von Willebrand disease

- Treatment

A
  • Humate
  • DDAVP
  • AMICAR
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15
Q

Hemophilia A vs. von Willebrand disease

- APTT

A
  • Hemophilia A: prolonged

- von Willebrand disease: prolonged (usually)

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16
Q

Hemophilia A vs. von Willebrand disease

- Bleeding time

A
  • Hemophilia A: normal

- von Willebrand disease: prolonged

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17
Q

Hemophilia A vs. von Willebrand disease

- Factor VIII activity

A
  • Hemophilia A: decreased

- von Willebrand disease: decreased (usually)

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18
Q

Hemophilia A vs. von Willebrand disease

- VIII: vWF Ag

A
  • Hemophlia A: ??

- von Willebrand disease: decreased

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19
Q

Hemophilia A vs. von Willebrand disease

- Platelet aggregation

A
  • Hemophilia A: ??

- von Willebrand disease: absent w/ ristocetin, normal w/ other agonists

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20
Q

Hemophilia A vs. von Willebrand disease

- PT

A
  • Hemophilia A:

- von Willebrand disease: normal

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21
Q

Congenital Afibrinogenemia

- Bleeding episodes

A

Bleeding w/ every hemostatic challenge

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22
Q

Congenital Afibrinogenemia

- Coagulation test results

A
  • PT: increased
  • APTT: increased
  • TCT: increased
  • Fibrinogen: absent
  • BT: increased
  • PLT aggregation: None (WHY??)
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23
Q

Congenital Afibrinogenemia

- Treatment

A

Cryoprecipitate

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24
Q

Congenital Hypofibrinogenemia

- Bleeding episodes

A

Mild to absent bleeding episodes

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25
Congenital Hypofibrinogenemia | - Coagulation test results (PT, APTT, TCT, fibrinogen)
- PT: increased - APTT: increased - TCT: increased - Fibrinogen: low (< 100 mg/dL)
26
Congenital Hypofibrinogenemia | - Treatment
Cryoprecipitate
27
Congenital Dysfibrinogenemia | - Bleeding episodes
Asymptomatic to mild bleeding episodes
28
Congenital Dysfibrinogenemia | - Coagulation test results (PT, APTT, TCT, reptilase)
- PT: variable - APTT: variable - TCT: increased - Reptilase: increased
29
Congenital Dysfibrinogenemia | - Treatment
Cryoprecipitate
30
Factor II deficiency | - How is it inherited?
?
31
Factor II deficiency | - Bleeding episodes
- Postoperative bleeding - Epistaxis - Menorrhagia - Easy bruising
32
Factor II deficiency | - Coagulation test results (PT, APTT)
- PT: increased | - APTT: increased
33
Factor II deficiency | - Treatment
PCC, raise to 75%
34
Factor V deficiency | - How is it inherited?
?
35
Factor V deficiency | - Bleeding episodes
Mild bleeding episodes
36
Factor V deficiency | - Coagulation test results (PT, APTT)
- PT: increased | - APTT: increased
37
Factor V deficiency | - Treatment
FFP, raise level to 75%
38
Factor VII deficiency | - How is it inherited?
?
39
Factor VII deficiency | - Bleeding episodes
Moderate to severe hemorrhagic symptoms including spontaneous hemorrhage, hemarthroses, postoperative bleeding
40
Factor VII deficiency | - Coagulation test results (PT, APTT)
- PT: increased | - APTT: normal
41
Factor VII deficiency | - Treatment
PPC*, FFP | * PCC not effective by itself, level too low in product
42
Factor X deficiency | - How is it inherited?
?
43
Factor X deficiency | - Bleeding episodes
Moderate to severe hemorrhagic symptoms including ecchymoses, CNS bleeding, excessive bleeding after childbirth
44
Factor X deficiency | - Coagulation test results (PT, APTT)
- PT: increased | - APTT: increased
45
Factor X deficiency | - Treatment
PCC, raise level to 75%
46
Factor XI deficiency | - How is it inherited?
?
47
Factor XI deficiency | - Bleeding episodes
Mild to moderate bleeding episodes
48
Factor XI deficiency | - Coagulation test results (PT, APTT)
- PT: normal | - APTT: increased
49
Factor XI deficiency | - Treatment
FFP, raise level to 75%
50
Factor XII deficiency | - How is it inherited?
?
51
Factor XII deficiency | - Bleeding episodes
Asymptomatic
52
Factor XII deficiency | - Coagulation test results (PT, APTT)
- PT: normal | - APTT: increased
53
Factor XII deficiency | - Treatment
None needed
54
Factor XIII deficiency | - How is it inherited?
?
55
Factor XIII deficiency | - Bleeding episodes
Bleeding w/ every hemostatic challenge including umbilical cord bleeding, delayed wound healing, intracranial hemorrhage
56
Factor XIII deficiency | - Coagulation test results (PT, APTT, urea solubility)
- PT: normal - APTT: normal - Abnormal urea solubility test
57
Factor XIII deficiency | - Treatment
Cryoprecipitate or FFP every 3 weeks
58
Fletcher Factor deficiency | - How is it inherited?
?
59
Fletcher Factor deficiency | - Bleeding episodes
Asymptomatic
60
Fletcher Factor deficiency | - Coagulation test results (PT, APTT)
- PT: normal | - APTT: increased
61
Fletcher Factor deficiency | - Treatment
None needed
62
Fitzgerald Factor deficiency | - How is it inherited?
?
63
Fitzgerald Factor deficiency | - Bleeding episodes
Asymptomatic
64
Fitzgerald Factor deficiency | - Coagulation test results (PT, APTT)
- PT: normal | - APTT: increased
65
Fitzgerald Factor deficiency | - Treatment
None needed
66
Types of bleeds for both Hemophilia A and B in infants
Excessive bleeding from circumcism
67
Types of bleeds for both Hemophilia A and B in toddlers
- Bleeding/large ecchymoses w/ teething, crawling, learning to walk/fall - Bleeding into mouth/pharynx → suffocation - Lifting may cause hematuria
68
Hemophilia A and B | - Bleeding episodes
Major disability is from crippling hemarthrosis - Bleeding into muscles/soft tissue - Circulation is blocked - Muscle atrophy/wasting - Gangrene may set in Major cause of death is intracranial hemorrhage
69
Hemophilia A and B | - Coagulation test results
- BT, PLT, PT: normal - APTT: prolonged - Mixing studies: PNP corrects - Confirm w/ Factor VIII or IX assay: decreased

Liz's Hemostasis/Coagulation (10 decks)

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