hemostasis/coagulation Flashcards

1
Q

3 major components to the hemostatic system

A

the vascular wall, platelets, and coagulation proteins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

3 steps of the platelet response to vascular injury (primary hemostasis)

A

adhesion, activation, aggregation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

platelet adhesion involves activation of a surface membrane receptor, _____, an adhesive protein, ____, and an appropriate surface, _____

A

glycoprotein Ib/IX
von Willebrand factor
subendothelial collagen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

in fibrin clot formation (secondary hemostasis), soluble coagulation proteins within plasma are activated to generate ____ in an ____ reaction

A

thrombin; amplification

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what does thrombin convert

A

thrombin converts fibrin to fibrinogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

fibrin adds ____ to the clot after fibrin monomers are covalently cross-linked by _____

A

stability; Factor XIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

sequence of the intrinsic pathway

A

activation of Factor XII by kallikrein
activation of Factor XI by Factor XIIa
Factor XIa activates Factor IX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

sequence of extrinsic pathway

A

activation of Factor VII by tissue factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

sequence of the common pathway

A

activation of X to Xa
conversion of prothrombin (II) to thrombin
conversion of fibrinogen (I) to fibrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

regulatory systems in secondary hemostasis

A

serine protease inhibitors (antithrombins)
protein C pathway (controls Va and VIIIa)
fibrinolysis (removes excess clot)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

antithrombins inhibit the activity of thrombin and other serine proteases (like ______) of the coagulation cascade by forming an inactive ______

A

factors IXa, Xa, XIa, and XIIa

enzyme-inhibitor complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

one of the best known antithrombins is _____, a molecule which, in the presence of _____, becomes activated so that it can form a complex with thrombin, thus destroying the ability of thrombin to participate in generation of _____

A

antithrombin III
heparin
fibrin monomers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

protein C system regulates the major cofactors of the coagulation cascade, _____

A

factors Va and VIIIa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

activated _____ is the major effector enzyme of the protein C system and ____ is a major cofactor

A

protein C; protein S

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

_____ promotes coagulation through resistance to enzymatic inactivation by the Protein C/S complex

A

Factor V Leiden mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

in the presence of fibrin, _____ can bind to _____ and convert it to the activate enzyme which breaks down fibrin monomers into fibrin degradation products

A

tissue plasminogen activator (TPA) ; plasminogen (active enzyme = plasmin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

common lab screening tests used to evaluate hemostasis

A
prothrombin time (PT)
international normalized ratio (INR)
partial thromboplastin time (PTT)
platelet count 
bleeding time
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

clinical manifestations of disorders of primary hemostasis include _____ and/or ______; laboratory manifestations include _____ and _____

A
mucocutaneous bleeding (petechial); bleeding associated with trauma
prolonged bleeding time; thrombocytopenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

clinical manifestations of disorders of secondary hemostasis include _____ and/or _____; laboratory manifestations include a _____ and/or _____

A

soft tissue bleeding; bleeding associated with trauma

prolonged PT and/or PTT; thrombin time

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

clinical manifestations of disorders of the regulatory system include _____ and/or ______; laboratory manifestations are often ____ in screening tests

A

soft tissue bleeding; bleeding associated with trauma

normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

congenital disorders associated with bleeding

A
von willebrand disease
hemophilia A (factor VIII deficiency)
hemophilia B (factor IX deficiency)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

two most frequent congenital bleeding disorders

A

von willebrand disease and hemophilia A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

von willebrand disease is an _____ disorder associated with production of decreased amounts of a _____ (quantitative abnormality) or production of a protein with _____ (qualitative abnormality), or both

A

autosomal dominant; normal protein; abnormal function

24
Q

predominant clinical manifestation of von willebrand disease is _____

A

mucocutaneous bleeding

25
Q

hemophilia A is an ______ disorder that is due to decreased production of _____

A

X-linked recessive; Factor VIII

26
Q

clinical hallmark of hemophilia A is _____

A

recurrent soft tissue bleeding

27
Q

hemophilia B is an ____ disorder that is due to decreased production of ____

A

X-linked recessive; Factor IX

28
Q

refers to a decrease in platelet count

A

thrombocytopenia

29
Q

bleeding due to thrombocytopenia typically appears as _____ and _____

A

petechial hemorrhages in skin and mucous membranes

30
Q

disorder characterized by immune-mediated destruction of platelets; autoantibodies are directed at the platelet membrane antigens

A

immune thrombocytopenic purpura (ITP)

31
Q

____ and ____ are common targets of immune thrombocytopenic purpura (ITP)

A

GPIb/IX ; IIb/IIIa

32
Q

thrombotic thrombocytopenic purpura (TTP) is characterized by _____ with formation of _____ throughout the circulation

A

intravascular platelet activation; platelet-rich microthrombi

33
Q

cause of TTP is due to a deficiency of a _____, called _____ that normally degrades very high molecular weight multimers of von willebrand factor

A

metalloproteinase; ADAMTS 13

34
Q

regulators of primary hemostasis (platelets)

A

NO, prostacyclin (PGI2), and ADPase

35
Q

as part of the regulation of coagulation, antithrombins inhibit _____, protein C inhibits _____ and plasmin does what?

A

serine proteases (like thrombin, factors 9a-12a)
cofactors Va and VIIIa
plasmin removes excess fibrin clot

36
Q

screens for activity of proteins in the extrinsic pathway (factors V, VII, II, X, and fibrinogen)

A

prothrombin time (PT)

37
Q

prothrombin time (PT) is a measurement of the time needed to ____ in the presence of added _____

A

form a clot; tissue thromboplastin (tissue factor)

38
Q

used to monitor patients on anticoagulation therapy (coumadin/warfarin)

A

international normalized ratio (INR)

39
Q

INR is a ratio of:

A

patient prothrombin time / control prothrombin time

40
Q

screens for activity within the intrinsic pathway (factors XII, XI, IX, VIII, X, V, II and fibrinogen)

A

partial thromboplastin time (PTT)

41
Q

PTT is a measurement of the time needed for plasma to form a clot in the presence of added ______ to activate _____

A

ground glass or kaolin (negatively charged activators of factor XII)
to activate contact-dependent Factor XII

42
Q

normal range for platelet count is _____

A

150,000 to 400,000 per microliter

43
Q

the term _____ refers to a decrease in platelet number; ____ and _____ denote an increase in platelet number

A

thrombocytopenia

thrombocytosis and thrombocythemia

44
Q

specialized tests of hemostasis

A

mixing studies
factor assays
circulating anticoagulant
platelet aggregation testing

45
Q

if mixing study corrects clotting time, a _______ is felt to be present

A

deficiency of some factor(s)

46
Q

if the mixing study does not correct the clotting time, an ____ is felt to be present

A

inhibitor (factor-specifc or lupus anticoagulent type)

47
Q

classification of bleeding disorders

A

primary vs. secondary hemostasis
congenital vs. acquired
regulatory disorder
mild vs severe

48
Q

classification of vWD

A

type I- quantitative deficiency (partial)
type II- qualitative deficiency
type III- quantitative deficiency (complete absence)

49
Q

most common hereditary cause for serious bleeding

A

hemophilia A

50
Q

mechanisms of thrombocytopenia

A
  • decreased platelet production
  • increased destruction
  • sequestration
  • congenital vs acquired
51
Q

ITP clinical features

A

petechial hemorrhages
gingival bleeding
ecchymoses (bruises)
bleeding with trauma or surgery

52
Q

factor VIII complex is composed of _____ and _____

A

vWF; factor VIII procoagulent

53
Q

lab features of vW disease

A

prolonged BT/PFA-100 and PTT

54
Q

leb features of hemophilia A

A

prolonged PTT and decreased factor VIII

55
Q

schistocytes in peripheral blood smear; increased bilirubin and LD, reflecting intravascular hemolysis

A

TTP (thrombotic thrombocytopenic purpura)