Coag Exam 2 Flashcards

1
Q

Coagulation Proteins (Factors)
In circulation:
Activated Form:
Purpose:

A

In circulation: inactive (zymogen)
Activated Form: interact to form fibrin Clot
Purpose: to reinforce the plt plug

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2
Q

Factor 1

A

Fribrinogen

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3
Q

Factor II

A

Prothrombin

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4
Q

Factor III

A

Tissue Factor (tissue thromboplastin)

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5
Q

Factor IV

A

Ionized calcium

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6
Q

Factor V

A

Labile Factor (proaccelerin)

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7
Q

Factor VII

A

Stable Factor (Serum prothrombin conversion accelerator SPCA), proconvertin

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8
Q

Factor VIII

A

Antihemopholic factor, factor VIII:C (coagulation portion)

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9
Q

Factor IX

A

Christmas Factor, Plasma thromboplastin component (PTC), antihemophilic factor B

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10
Q

Factor X

A

Stuart-Prower factor

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11
Q

Factor XI

A

Plasma Thromboplastin Antecedent (PTA)

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12
Q

Factor XII

A

Hageman factor (contact factor)

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13
Q

Factor XIII

A

Fibrin-stabilizing factor (FSF)

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14
Q

Fitzgerald factor

A

High-Molecular Weight Kininogen (HMWK)

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15
Q

Fletcher factor

A

Prekallikrein

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16
Q

Vitamin K dependent

A

Factors: 2, 7, 9 and 10

Protein C and S

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17
Q

effects of Coumarin, Coumadin and Warfarin

A
  • inhibit Vit K reduction yielding inactive factors

- only active in vivo (unlike heparin)

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18
Q

Fibrinogen or Thrombin sensitive proteins

A

1, 5, 8c and 13

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19
Q

Thrombin acts on all factors in the fibrinogen group
Enhances activity of:
Activates factor:
Converts:

A

Enhances activity of factors 5 and 8c
Activates factor 8
Converts fibrinogen to fibrin

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20
Q

PT is measured by which pathway?

A

Extrinsic

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21
Q

APTT is measured by which pathway?

A

Intrinsic

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22
Q

3 disorders of Fibrinogen

A

Afibrinogenemia, hypofibriogenemia and and dysfibrinogenemia

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23
Q

What is afibrinogenemia?

A

quantitative disorder

lack of synthesis in liver

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24
Q

What is hypofibrinogenemia?

A

low amounts of fibrinogen and is generally asymtomatic

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25
Q

What is dysfibrinogenemia?

A

abnormal fibrinogen structure and function

symptoms: post-traumatic or postoperative bleeding of mucosal tissues

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26
Q

What does factor 8 do?

A

functions as a catalyst, forming bonds between proteins (fibrin monomers, fibronectin, collagen, alpha 2-inhibitor)

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27
Q

What is factor 8 characterized by?

A

initial stoppage of bleeding then recurrence of bleeding more than 36 hours after event

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28
Q

Lab results and treatment for factor 8 deficiency

A

Normal: PT, APTT, TT, BT, fibrinogen, plt count
Low levels: F8 detected by 5M urea test
Treatment: FFP or cyro

29
Q

3 acquired disorders that are secondary to other pathological events

A

DIC, primary fibrinolysis and liver disease

30
Q

What is DIC?

A

disseminated idiopathic coagulation
Classification: consumption coagulopathy (plt and factors depleted)
Physiologic effects: plasminogen activated, results in increased FDP’s and possible RBC fragmentation

31
Q

Lab results for DIC

A

Plt count: decreased
Fibrinogen: decreased
PT, APTT, and TT: prolonged
FDP or d Dimer: elevated

32
Q

Cause of DIC

A

pathway activation
Extrinsic: large amounts of tissue factor entering blood stream
Intrinsic: events that damage vascular endothelium exposing collagen

33
Q

Treatment for DIC

A

FFP, plts, cryo, low molecular weight heparin to break cycle and treat symptoms, remove stimulus

34
Q

What is Primary Fibrinolysis?

A

symptomatically similar to DIC
results from increased levels of plasmin
Patient presentation: diffuse hemorrhages due to increased plasmin fibrinolytic activity and will initially form a clot that dissolves in 1-2 hours

35
Q

Causes of primary fibrinolysis

A

cirrhosis, shock, metastatic CA of prostate, injury to urinary tract or leakage of urokinase from urine into tissues

36
Q

Lab tests for fibrinolysis

A
Fibrinogen: decreased
PT and APTT: prolonged
FDP: increased
d-Dimer: normal
Plt count: normal
RBCs: normal, no fragmentation
37
Q

In liver disease a decrease in what factor happens first and why?

A

Factor 7 because it has the shortest half life

38
Q

What is seen in 1/3 of chronic liver disease patients?

A

Thrombocytopenia due to spleen sequestration secondary to congestive spenomegaly

39
Q

What is the most common hereditary coag disorder and what is it’s inheritance pattern?

A

Hemophilia A ( Factor 8:C)
X-linked recessive-factor 8:C on X
*males more effected

40
Q

_________ or ________ develop in 10 to 15% of factor 8:C deficiencies.

A

alloantibodies or inhibitors

41
Q

Symptoms of Hemophillia A?

A
Hemarthrosis
Hematuria
Intracranial bleeds
Hematomas
Spontaneous hemorrhage
42
Q

Lab Findings

1-BT
2-PT and TT
3-APTT
4-Factor 8:C activity
5-vWF: Ag
6-Plt function assays 
7-mixing studies
A
1-Normal
2-Normal
3-Prolonged-Factor 8:C level< 20%
4-Low to absent
5-Normal
6-Normal
7-mixing studies do not correct with pooled plasma
43
Q

Treatment for Hemophilia A?

A

Replacement therapy or Cryo

44
Q

1-Hemophilia B?
2-aka
3-inheritance pattern
4-activated by

A

1-Factor 9 deficiency
2-Christmas disease or Plasma thromboplastin component
3-sex linked- less common than Hemophilia A
4-XIa, Ca2+,via TF and Factor VIIa or Russell Viper Venom

45
Q

Treatment Hemophilia B

A
  • regular infusion with FFP (contains active F9)
  • prothrombin complex ( F2,7,9,10)
  • or F 9 concentrates
46
Q

Hemophilia B Lab Tests
1-PTT,TT,BT
2-APTT
3-mixing studies

A

1-Normal
2-Increased
3-corrected with normal pool or aged serum

47
Q

1-Hemophilia C
2-aka
3-found in what population

A

1- Factor 11 deficiency
2-Rosenthal syndrome
3-Ashkenazi jewish population

48
Q

Is replacement therapy necessary?

A

no unless patient is schedule for surgery- then they need FFP to increase factor assay level to 20-30%

49
Q

Factor 11 levels >120% at risk for ?

A

thrombosis

50
Q

Hemophilia C Lab Tests
1-PT,BT,TT
2-APTT
3-Factor 11 assay

A

1-normal
2-Increased
3-decreased (normal 70-130%)

51
Q

Factor 7 deficiency
1-A.K.A.
2-inheritance pattern

A

1-procoverntin

2-rare auto recessive

52
Q

Symptoms of Factor 7 deficiency?

A
  • deep muscle hepatomas
  • joint hemorrhage
  • epistaxis
  • menorrhagia
53
Q

Lab Tests for Factor VII Deficiency
1-APTT,TT,BT
2-PT

A

1-Normal

2-prolonged (fully corrected with Russells Viper venom (activates FX) and mixing with aged serum

54
Q

Factor X Defeciency

1-AKA
2-inheritance pattern

A

1-Stewart Prower Factor

2-rare auto recessive at any age

55
Q

Normal reference range for Factor X?

A

50-150%

56
Q

Factor X lab test?

1-PT,APTT
2-TT,BT
3-Stypfentime or RVVT

A

1-abnormal
2-normal
3-prolonged

57
Q

Treatment of Factor 10?

A

FFP or Prothrombin concentrates

58
Q

Factor V

1-AKA
2-Catalyst in?
3-inheritance
4-normal range?
5-Acquired FV?
A

1-labile factor ( because of rapid deterioration in plasma at room temp
2-conversion of conversion of FII to IIa
3-Rare autosomal recessive
4-50-150%
5-specific Ab acquired after childbirth or use of fibrin glue in surgery (associated with liver disease, carcinoma, TB, DIC, etc…

59
Q

Lab tests for Factor V deficiency?

1-specimens
2-APTT,PT
3-TT
4-

A

1-Specimens must be diluted (platelet poor)
2-prolonged
3-Normal
4-FFP

*not cry because there is not enough FV in concentrate

60
Q

Factor II Deficiency

1-AKA
2-delays what?

A

1-prothrombin

2-delays generation of thrombin-hemorrhagic symptoms

61
Q

What is the normal reference range for Factor II and i has the longest what?

A
  • 50-150%

- has the longest 1/2 life of the Vitamin K factors

62
Q

Factor II is converted to thrombin by what?

A

the action of Factor Xa and Va + Platelet Factor 3 + Ca2++ ( known as prothrombininase complex, assembled on plot surface

63
Q

Factor II (Hypoprothrombinemia)

Lab tests
1-PT,APTT
2-TT
3-Diagnosis dependent on?

A

1-prolonged
2-Normal
3-….activity assay or antigenic concentration of prothrombin or both ( both should be decreased)

64
Q

Factor II (Dysprothrombinemia)

A

structural defect that causes impaired activity

65
Q

Treatment for Factor II issues

A
  • FFP

- prothrombin complex factor concentrates

66
Q
Factor II prothrombin mutations (G20210A).
1-occurs where?
2-2nd most common cause ?
3-restricted to what population?
4-Must be detected via?
5-identified as risk factors for
A
1-chromosome 11
2-of inherited thrombophilia
3-caucasion
4-DNA analysis by PCR
5-MI in young women, stroke in young patients
67
Q

Hageman (Factor 12 deficiency)

1-aka
2-Lab tests (PT,APTT, F12 analysis)
3-normal reference range

A
1-Hageman trait
2-PT- Normal
  -APTT-prolonged
  -Factor 12 analysis-decreased
3-70-140%
68
Q

Prekallikrein (Fletcher) deficiency

1-clinical bleeding?
2-prediposition to race?
3-APTT
4-contact activation time in APTT kaolin-like reagents changes?

A

1-no
2-none
3-marked prolonged
4-incubation time intervals will progressively shorter the APTT