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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
What does tissue plasminogen activator work on
fibrin bound plasmin at site of thrombus
26
what inhibits tissue plasminogen activator
PAl-1
27
what is the purpose of urokinase
prevents fibrin clots in renal collecting ducts
28
what inhibits urokinase
PAI-1
29
What is unfractionated heparin
a mix of glycosamines from pig intestines indirect anticoagulant that accelerates the action of antithrombin monitored via PTT or anti-Xa
30
What is low molecular weight heparin
fractionated heparin monitored through anti-Xa only
31
What is fondaparinux
synthetic formulation of active sequence of heparin increases antithrombin activity monitored through anti-Xa
32
What is coumadin
acts on liver to impair production of vitamin K factors monitored via PT
33
What is dabigatran
a direct thrombin inhibitor monitored via TT
34
What is rivaroxaban and apixaban
inhibits FX monitored via anti-Xa
35
What is the testing window for PTT
4 hours
36
What is the testing window for PT
24 hours
37
What are interferences in coagulation testing
clots underfilled heparin hemolysis prolonged tourniquet application lipemia cold storage heat
38
What is the principle of mechanical end-point clot detection
measures the variation in amplitude of the oscillation of a steel ball as clot is formed ball moves less
39
What is the principle of photo-optical end-point clot detection
as the clot is formed the change in transmittance is detected by the instrument
40
What is the principle of the chromogenic end-point detection
a chromogenic labelled oligopeptide is used to detect activity of factors, when the oligopeptide is cleaved by the factor it puts of a signal
41
What is the principle of light -absorbance end-point detection
when microparticles agglutinate there is a decrease in transmittance which causes an increased absorbance proportional to the antigen of interest
41
What reagents are used for PT
tissue thromboplastin (recombi) and calcium
42
What causes prolonged PT
abnormalities/deficienceies in the extrinsic and common pathway vitamin K deficency
43
What causes decreased PT
myocardial infarction, multiple myeloma, drug therapy
44
What is the reference range for PT
0.8-1.2
45
What is the therapeutic range for PT
2.0-3.5
46
What is the critical value for PT
>5.0
47
What are the reagents used for PTT
calcium, negatively charged contact activator
48
What cause prolonged PTT
abnormailties/deficiencies in the intrinsic and common pathway circulating anticoagulants liver disease, vitamin K deficiency
49
What is the reference range for PTT
26-38 sec
50
What is the therapeutic range for PTT
low: 59-83 high: 84-88
51
What is the critical value for PTT
>120 sec
52
What can cause decreased fibrinogen
congenital afibrinogenemia. DIC, systemic fibrinolysis, pancreatitis, severe hepatic dysfunction
53
What is the critical value for fibrinogen
<1.0g/L
54
What is the principle of a mixing study
patient plasma is mixed 1:1 with normal plasma before testing to determine if the normal plasma corrects any prolonged PT/PTT results
55
How do we remove heparin from a sample
hepzyme
56
What is an inhibitor screen
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
What is the purpose of platelet aggregation studies
assess platelet function
58
What occurs in a platelet aggregation study
platelet rich plasma is stirred monitoring for changes in light transmission as various agonists are added
59
What are the platelet aggregation study results for VWF disease
ADP, collagen, epinephrine, AA are all normal Ristocetin is variable based on typw I- abnormal 2A- abnormal 2B- normal 3- absent
60
What are the platelet aggregation study results for bernard soulier
ADP, collagen, epinephrine, AA are all normal ristocetin is absent
61
What are the platelet aggregation study results for Glanzmans
ADP, collagen, epinephrine, AA are all absent ristocetin demonstrates aggregation followed by disaggregation
62
What are the platelet aggregation study results for storage pool diseaseq
ADP, collagen, epinephrine, AA are all variable with collagen ristocetin is normal
63
What are the platelet aggregation study results for alpha granule deficency
ADP, collagen, epinephrine, AA are all decreased ristocetin is normal
64
What does the ristocetin cofactor assay test
ensures all coagulative components are present if aggregation occurs then the patient has VWF
65
What does reptilase time test
if the patient has adequate fibrinolysis prolonged - hypofinbrinogenemia or dysfibrinogenemia
66
What can cause factor deficiencies
liver disease, vitamin K deficiency, auto immune
67
What is vonwillebrand disease
a congenital bleeding disorder with auto dominant inheritance pattern that causes a deficiency of VWF which in turn causes decreased FVIII
68
What are test results for vonwillebrand disease
PT/PTT normal to prolonged PLT count normal Bleeding time prolonged vWF assay low FVIII assay low Ristocetin aggregation variable Ristocetin cofactor negative
69
What is hemophilia A
a congenital bleeding disorder with X-linked recessive inheritance that effects FVIII
70
What are test results for hemophilia A
PT normal PTT prolonged TT prolonged bleeding time normal
71
What is hemophilia B
an X-linked bleeding disorder that effects FIX
72
What are test results for hemophilia B
PT normal PTT prolonged bleeding time normal
73
What is Hemophilia C
auto dominant bleeding disorder that effects FXI
74
What are test results for hemophilia C
PT normal PTT prolonged TT normal bleeding time normal
75
What is hypofibrinogenemia
auto-recessive or acquired disorder leading to low levels of fibrinogen
76
What are test results for hypofibrinogenemia
PT abnormal PTT abnormal TT abnormal FIB abnormal BT abnormal
77
What is afibrinogenmia
an auto recessive disorder that causes the absense of functionally detectable fibrinogen in plasma
78
What are test results for afibrinogenmia
PT abnormal PTT abnormal TT abnormal FIB abnormal BT abnormal PLT adhesion/aggregation abnormal
79
What is dysfibrinogenemia
auto dominant disorder that cause the alteration of the structure of fibrinogen effecting the interaction of fibrinogen with other molecules
80
What are test results for dysfibrinogenemia
PT normal PTT normal BT normal TT prolonged PLT function normal reptilase time very prolonged
81
What are contact factor deficiencies
auto recessive disorder effecting FXII, HMWK, prekallekrein
82
What are test results for contact factor deficiencies
PT normal TT normal BT normal PTT abnormal Factor assay decerased
83
What is factor XIII deficiency
a deficience that effects FXIII keading to decreased cross linking
84
What are test results for factor XIII deficiencey
factor assay abnormal clot dissolving in 5M urea or 1% monochloroacetic acid
85
What is APC resistance
auto dominant disorder causing a defect in FV so it cannot be bound by APC (FV cannot be regulated)
86
What is Prothrombin G20210A mutation
a mutation in prothrombin causing elevated plasma prothrombin levels
87
What is lupus anticoagulant
a nonspecific inhibitor which prolongs PTT by binding to the commercial phospholipid reagent that causes risk of thrombosis
88
What is the dilute russel viper venom time
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
What is megakaryocyte hypoplasia
a decrease in megakaryocytes which in turn causes a decrease in PLT
90
What is ITP
IgG antibodies attach to PLT causing them to be removed by the spleen. PLT are often large and abnormal due to accelerated production
91
What is neonatal thrombocytopenia
mother develops antibodies to fetal PLT
92
What is drug indiced thrombocytopenia
caused by salicylates, acetaminiohen, penicillin, sulfa drugs, quinidine, phenobarbital
93
What is heparin induced thrombocytopenia
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
What is Bernard Soulier Syndrome
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
What are lab results for bernard soulier syndrome
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
What is Glazmanns thrombasthenia
an autosomal recessive disorder that causes severe hemorrhage. There is an absence of GPIIb/IIIa receptors on the platelets causing defective aggregation
97
What are lab tests for Glazmanns thrombasthenia
PT/PTT normal PLT count/morph normal bleeding time prolonged PLT affregation normal with ristocetin
98
What is dense granule deficiency
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
What is alpha granule deficency
an autosomal recessive disorder causing the absence of alpha granules leading to an agranular appearance
100
What are lab tests for alpha granule deficency
PT/PTT normal PLT count decreased agranular PLT bleeding time prolonged

Hematology (7 decks)

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