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Hematology > Coagulation > Flashcards

Coagulation Flashcards

1
Q

What is primary hemostasis

A

the activation and action of platelets in the formation of the initial clot or platelet plug

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2
Q

What initiates primary hemostasis

A

vascular injury which causes the release of procoagulant factors

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3
Q

What are the anticoagulant properties of the endothelium

A

secretion of prostacyclin, nitric oxide, heparin sulfate, tissue factor pathway inhibitor, tissue plasminogen activator
smooth continuous surface inert to PLT and coag factors
expression of continuous surface inert to PLT and coagulation factors
expression of cell membrane thrombomodulin

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4
Q

What does prostacyclin

A

platelet inhibitor

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5
Q

What does nitric oxide do

A

vascular relaxing

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6
Q

What are the procoagulant properties of the endothelium

A

exposure of collagen
vasoconstriction caused by harm and factors released by the platelets
secretion of VWF
sub-endothelial cells contain TF

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7
Q

What does thromboxane A2 do

A

causes calcium to be released and promotes PLT aggregation and vasoconstriction

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8
Q

What are the purpose of dense PLT granules

A

fuse with plasma membrane to secrete contents
contain ADP, ATP, Ca, Mg, serotonin

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9
Q

What are the purpose of alpha PLT granules

A

secrete contents into surface-connected canalicular system that releases contents to external environment
contains PF4, B thromoglobulin, platelet derived growth factor, endothelial growth factor, transforming growth factor, factors V, XI, VWF, fibrinogen

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10
Q

What is used to monitor the intrinsic pathway

A

PTT

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11
Q

What is used to monitor the extrinsic pathway

A

PT

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12
Q

What are the vitamin K dependant factors

A

II, VII, IX, X

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13
Q

What factors are in the common pathway

A

I, II, XIII

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14
Q

What factors are in the extrinsic pathway

A

III, VII, X

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15
Q

What factors are in the intrinsic pathway

A

VIII, IX, X, XI, XII

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16
Q

What is a zymogen

A

inactive form of an enzyme

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17
Q

What is a serine protease

A

active form of the clotting factor whose activity depends on the amino acid serine at activation site

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18
Q

What factors are the serine proteases

A

kallikrein, II, VII, IX, X, XI, XII, plasmin, protein C

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19
Q

What is the role of pf3

A

during platelet activation they are converted to arachidonic acid and the thromboxane A2 which promotes the release of calcium which allows propagation of the cascade

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20
Q

What is the role of VWF

A

stabilizes factor VIII

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21
Q

What are regulators of coagulation

A

fibrinolytic system
regulatory mechanisms of coagulation - tissue factor pathway inhibitor, protein c system, anti-thrombin, protein Z dependent protease inhibitor

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22
Q

what activates plasminogen

A

tissue plasminogen activator

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23
Q

what does plasminogen act on

A

fibrin

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24
Q

What inhibits plasminogen

A

alpha-2 antiplasmin

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25
Q

What does tissue plasminogen activator work on

A

fibrin bound plasmin at site of thrombus

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26
Q

what inhibits tissue plasminogen activator

A

PAl-1

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27
Q

what is the purpose of urokinase

A

prevents fibrin clots in renal collecting ducts

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28
Q

what inhibits urokinase

A

PAI-1

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29
Q

What is unfractionated heparin

A

a mix of glycosamines from pig intestines
indirect anticoagulant that accelerates the action of antithrombin
monitored via PTT or anti-Xa

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30
Q

What is low molecular weight heparin

A

fractionated heparin
monitored through anti-Xa only

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31
Q

What is fondaparinux

A

synthetic formulation of active sequence of heparin
increases antithrombin activity
monitored through anti-Xa

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32
Q

What is coumadin

A

acts on liver to impair production of vitamin K factors
monitored via PT

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33
Q

What is dabigatran

A

a direct thrombin inhibitor
monitored via TT

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34
Q

What is rivaroxaban and apixaban

A

inhibits FX
monitored via anti-Xa

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35
Q

What is the testing window for PTT

A

4 hours

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36
Q

What is the testing window for PT

A

24 hours

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37
Q

What are interferences in coagulation testing

A

clots
underfilled
heparin
hemolysis
prolonged tourniquet application
lipemia
cold storage
heat

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38
Q

What is the principle of mechanical end-point clot detection

A

measures the variation in amplitude of the oscillation of a steel ball
as clot is formed ball moves less

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39
Q

What is the principle of photo-optical end-point clot detection

A

as the clot is formed the change in transmittance is detected by the instrument

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40
Q

What is the principle of the chromogenic end-point detection

A

a chromogenic labelled oligopeptide is used to detect activity of factors, when the oligopeptide is cleaved by the factor it puts of a signal

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41
Q

What is the principle of light -absorbance end-point detection

A

when microparticles agglutinate there is a decrease in transmittance which causes an increased absorbance proportional to the antigen of interest

41
Q

What reagents are used for PT

A

tissue thromboplastin (recombi) and calcium

42
Q

What causes prolonged PT

A

abnormalities/deficienceies in the extrinsic and common pathway
vitamin K deficency

43
Q

What causes decreased PT

A

myocardial infarction, multiple myeloma, drug therapy

44
Q

What is the reference range for PT

A

0.8-1.2

45
Q

What is the therapeutic range for PT

A

2.0-3.5

46
Q

What is the critical value for PT

A

> 5.0

47
Q

What are the reagents used for PTT

A

calcium, negatively charged contact activator

48
Q

What cause prolonged PTT

A

abnormailties/deficiencies in the intrinsic and common pathway
circulating anticoagulants
liver disease, vitamin K deficiency

49
Q

What is the reference range for PTT

A

26-38 sec

50
Q

What is the therapeutic range for PTT

A

low: 59-83
high: 84-88

51
Q

What is the critical value for PTT

A

> 120 sec

52
Q

What can cause decreased fibrinogen

A

congenital afibrinogenemia. DIC, systemic fibrinolysis, pancreatitis, severe hepatic dysfunction

53
Q

What is the critical value for fibrinogen

A

<1.0g/L

54
Q

What is the principle of a mixing study

A

patient plasma is mixed 1:1 with normal plasma before testing to determine if the normal plasma corrects any prolonged PT/PTT results

55
Q

How do we remove heparin from a sample

A

hepzyme

56
Q

What is an inhibitor screen

A

tests to see if an inhibitor is delayed or immediate. Patient plasma is mixed and tested then incubated at 37 and tested alongside unmixed patient plasma that was also incubated

57
Q

What is the purpose of platelet aggregation studies

A

assess platelet function

58
Q

What occurs in a platelet aggregation study

A

platelet rich plasma is stirred monitoring for changes in light transmission as various agonists are added

59
Q

What are the platelet aggregation study results for VWF disease

A

ADP, collagen, epinephrine, AA are all normal
Ristocetin is variable based on typw
I- abnormal
2A- abnormal
2B- normal
3- absent

60
Q

What are the platelet aggregation study results for bernard soulier

A

ADP, collagen, epinephrine, AA are all normal
ristocetin is absent

61
Q

What are the platelet aggregation study results for Glanzmans

A

ADP, collagen, epinephrine, AA are all absent
ristocetin demonstrates aggregation followed by disaggregation

62
Q

What are the platelet aggregation study results for storage pool diseaseq

A

ADP, collagen, epinephrine, AA are all variable with collagen
ristocetin is normal

63
Q

What are the platelet aggregation study results for alpha granule deficency

A

ADP, collagen, epinephrine, AA are all decreased
ristocetin is normal

64
Q

What does the ristocetin cofactor assay test

A

ensures all coagulative components are present
if aggregation occurs then the patient has VWF

65
Q

What does reptilase time test

A

if the patient has adequate fibrinolysis
prolonged - hypofinbrinogenemia or dysfibrinogenemia

66
Q

What can cause factor deficiencies

A

liver disease, vitamin K deficiency, auto immune

67
Q

What is vonwillebrand disease

A

a congenital bleeding disorder with auto dominant inheritance pattern that causes a deficiency of VWF which in turn causes decreased FVIII

68
Q

What are test results for vonwillebrand disease

A

PT/PTT normal to prolonged
PLT count normal
Bleeding time prolonged

vWF assay low
FVIII assay low

Ristocetin aggregation variable

Ristocetin cofactor negative

69
Q

What is hemophilia A

A

a congenital bleeding disorder with X-linked recessive inheritance that effects FVIII

70
Q

What are test results for hemophilia A

A

PT normal
PTT prolonged
TT prolonged
bleeding time normal

71
Q

What is hemophilia B

A

an X-linked bleeding disorder that effects FIX

72
Q

What are test results for hemophilia B

A

PT normal
PTT prolonged
bleeding time normal

73
Q

What is Hemophilia C

A

auto dominant bleeding disorder that effects FXI

74
Q

What are test results for hemophilia C

A

PT normal
PTT prolonged
TT normal
bleeding time normal

75
Q

What is hypofibrinogenemia

A

auto-recessive or acquired disorder leading to low levels of fibrinogen

76
Q

What are test results for hypofibrinogenemia

A

PT abnormal
PTT abnormal
TT abnormal
FIB abnormal
BT abnormal

77
Q

What is afibrinogenmia

A

an auto recessive disorder that causes the absense of functionally detectable fibrinogen in plasma

78
Q

What are test results for afibrinogenmia

A

PT abnormal
PTT abnormal
TT abnormal
FIB abnormal
BT abnormal
PLT adhesion/aggregation abnormal

79
Q

What is dysfibrinogenemia

A

auto dominant disorder that cause the alteration of the structure of fibrinogen effecting the interaction of fibrinogen with other molecules

80
Q

What are test results for dysfibrinogenemia

A

PT normal
PTT normal
BT normal
TT prolonged
PLT function normal
reptilase time very prolonged

81
Q

What are contact factor deficiencies

A

auto recessive disorder effecting FXII, HMWK, prekallekrein

82
Q

What are test results for contact factor deficiencies

A

PT normal
TT normal
BT normal
PTT abnormal
Factor assay decerased

83
Q

What is factor XIII deficiency

A

a deficience that effects FXIII keading to decreased cross linking

84
Q

What are test results for factor XIII deficiencey

A

factor assay abnormal
clot dissolving in 5M urea or 1% monochloroacetic acid

85
Q

What is APC resistance

A

auto dominant disorder causing a defect in FV so it cannot be bound by APC (FV cannot be regulated)

86
Q

What is Prothrombin G20210A mutation

A

a mutation in prothrombin causing elevated plasma prothrombin levels

87
Q

What is lupus anticoagulant

A

a nonspecific inhibitor which prolongs PTT by binding to the commercial phospholipid reagent that causes risk of thrombosis

88
Q

What is the dilute russel viper venom time

A

used to detect lupus anticoagulant by initiating the coagulation cascade by activating FX. confirm reagent is rich in phospholipid and neutralizes LAC shortening the time

89
Q

What is megakaryocyte hypoplasia

A

a decrease in megakaryocytes which in turn causes a decrease in PLT

90
Q

What is ITP

A

IgG antibodies attach to PLT causing them to be removed by the spleen. PLT are often large and abnormal due to accelerated production

91
Q

What is neonatal thrombocytopenia

A

mother develops antibodies to fetal PLT

92
Q

What is drug indiced thrombocytopenia

A

caused by salicylates, acetaminiohen, penicillin, sulfa drugs, quinidine, phenobarbital

93
Q

What is heparin induced thrombocytopenia

A

an adverse effect of UFH that causes an IgG antibody to be made. The antibody complexes with heparin and then the complex binds to platelet receptors which leads to PLT activation

94
Q

What is Bernard Soulier Syndrome

A

dective platelet function leads to nosebleeds, bruising, and bleeding. Manifested in infancy or childhood it is a autosomal recessive disorder in which the GPIb complex is missing which means PLT cant bind to VWF causing defective adhesion

95
Q

What are lab results for bernard soulier syndrome

A

PT/PTT normal
PLT count low
bleeding time prolonged
PLT aggregation shows no response to ristocetin
PLT have cytoplasmic vacuoles visible on electron microscope

96
Q

What is Glazmanns thrombasthenia

A

an autosomal recessive disorder that causes severe hemorrhage. There is an absence of GPIIb/IIIa receptors on the platelets causing defective aggregation

97
Q

What are lab tests for Glazmanns thrombasthenia

A

PT/PTT normal
PLT count/morph normal
bleeding time prolonged
PLT affregation normal with ristocetin

98
Q

What is dense granule deficiency

A

can be associated with albinism or not. Dense granule membranes are present but unable to pack granule contents. Usually only cause bruising though it does effect PLT aggregation studies

99
Q

What is alpha granule deficency

A

an autosomal recessive disorder causing the absence of alpha granules leading to an agranular appearance

100
Q

What are lab tests for alpha granule deficency

A

PT/PTT normal
PLT count decreased
agranular PLT
bleeding time prolonged

Hematology (7 decks)

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