Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

gen-path 2 > Coagulation > Flashcards

Coagulation Flashcards

1
Q

Primary hemostasis: name the membrane receptor, Adhesive protein, and appropriate surface

A

Membrane receptor: Glycoprotein 1b

Adhesive protein: Von Willebrand factor

Appropriate surface: Subendothelial matrix (collagen)

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

name the events that occur during platelet activation:

A
  • platelets are activated by binding to vWF

Secretion – alpha-granules and dense bodies (2nd messengers)

  • Activation of GP IIb/IIIa receptor
  • Shape change – from rounded discs to flat plates
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what protein is responsible for holding platelets together during aggregation?

A

fibrinogen

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

what steps occur during secondary hemostasis?

A

Secondary hemostasis = fibrin clot formation

Formation of thrombin

Formation of fibrin clot
- with crosslinking of fibrin monomers by Factor XIIIa

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

what molecules will inhibit secondary hemostasis?

A

Serine protease inhibitors (antithrombin)

Protein C pathway (controls Va and VIIIa)

Fibrinolytic system (removes excess clot- TPA)

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

what are the components of Anti-thrombin?

A

Serine Protease

+

SERPIN

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

Serine protease + SERPIN forms what?

A

Enzyme-Inhibitor Complex

(antithrombin

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

what molecules does serine protease (antithrombin) inhibit?

A

Thrombin

factors IXa, Xa, XIa, and XIIa

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

Activated Protein C (APC) plus Protein S serve to inactivate cofactors __________

A

Va and VIIIa

the major Cofactors in coagulation cascade

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

Protein C or Protein S deficiencies result in _________________

A

hypercoaguable states

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

what are the effects of a Factor V Leiden mutation?

A

promotes coagulation

resistance to enzymatic inactivation by the Protein C/S complex

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

In the presence of fibrin, _________________ can bind to plasminogen and convert it to an active enzyme, plasmin

A

tissue plasminogen activator (TPA)

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

Plasmin breaks down previously cross-linked fibrin monomers into what?

A

fibrin degradation products (FDP)

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

Uncontrolled activation of plasmin can result in bleeding complications as ____________ and _____________

A

fibrinolysis and fibrin(ogen)olysis

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

___________ inhibits serine proteases

A

antithrombin

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

protein C inhibits ________

A

cofactors Va and VIIIa

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

what are the screening tests of hemostasis?

A

Prothrombin Time (PT)
Partial Thromboplastin Time (PTT)
Platelet count
Bleeding time (BT)/PFA-100

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

The most important part of defining the cause of a bleeding disorder is _______________

A

a careful clinical history

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Prothrombin Time (PT) screens for activity of proteins in the __________ pathway

A

extrinsic

factors V, VII, II, X and fibrinogen

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

Partial thromboplastin time (PTT) screens for activity within the _________ pathway

A

intrinsic

factors XII, XI, IX, VIII… X, V, II and fibrinogen

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

what is the normal range for platelet counts?

A

normal range is generally 150,000 to 400,000/µL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what is thrombocytopenia? thrombocytosis? thrombocythemia?

A
  • thrombocytopenia refers to a decrease in platelet number

- thrombocytosis and thrombocythemia denote an increase in platelet number

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

what do the results of a positive mixing test show? what about a negative test?

A
  • If the mixing study corrects the clotting time, a deficiency of some factor(s) is felt to be present
  • If the mixing study does not correct the clotting time, an inhibitor is felt to be present, either factor-specific or lupus anticoagulant type
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

how are bleeding disorders classified?

A

Congenital vs. Acquired
Mild vs. Severe
Primary vs. Secondary Hemostasis
Regulatory Disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

what are the CLINICAL manifestations of Disorders of Primary Hemostasis?

A 
Mucocutaneous bleeding (vWF disease)
Excessive bleeding with trauma (vWF-d, Hemophilia A &B)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

what are the lab findings of Disorders of Primary Hemostasis?

A
  • Prolonged BT/PFA-100

- Thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what are the clinical manifestations of Disorders of secondary Hemostasis?

A
  • Soft tissue bleeding

- Excessive bleeding with trauma

28
Q

what are the lab findings of Disorders of secondary Hemostasis?

A

Prolonged PT and PTT

Prolonged TT (thrombin time)

29
Q

what are the clinical manifestations of regulatory disorders of Hemostasis?

A

Soft tissue bleeding

Excessive bleeding with trauma

30
Q

what are the lab findings of regulatory disorders of Hemostasis?

A

Normal PT and PTT
Normal bleeding time
Normal platelet count

31
Q

list the characteristics of von Willebrand disease:

A

Autosomal dominant disorder (usually)

Due to abnormalities of vWF

Abnormality may be quantitative, qualitative or both

Mucocutaneous bleeding is the dominant clinical manifestation

32
Q

what is the Factor VIII Complex?

A

vWF + factor VIII procoagulant

33
Q

what is the most commonly inherited bleeding disorder?

A

von Willebrand disease

  • effects 1% of population
34
Q

what are the 3 classes/types of von Willebrand disease?

A

Type 1: Quantitative deficiency (partial)
Type 2: Qualitative deficiency
Type 3: Quantitative deficiency (total)

35
Q

clinical manifestations of von Willebrand disease:

A 
*mucosal bleeding (NOT SEEN in hemophilia A)
Epistaxis (nosebleeds)
Ecchymoses (>1cm bruises)
Bleeding with trauma or surgery
Symptoms improve after adolescence
36
Q

in a patient with vWD, what lab tests would be normal? which would be elevated/prolonged? which would decrease?

A

PT & platelet count = normal

BT/PFA-100: Prolonged
*PTT: prolonged
*VIII: Decreased
vWF:ag: Decreased
vWF:Rcof: Decreased
37
Q

treatments for vWD include what?

A

Desmopressin
Antifibrinolytic agents
Factor VIII concentrates
Cryoprecipitate

38
Q

list the general characteristics of Hemophilia A:

A

Sex-linked recessive disorder

Deficiency of factor VIII

Recurrent soft tissue bleeding

Symptoms usually start in early childhood

39
Q

clinical characteristics of Hemophilia A:

A 
Hemarthrosis
Soft tissue bleeding
Excessive bleeding with trauma
Intramuscular hematomas
Intracerebral hemorrhage
Bleeding into other tissues
40
Q

which lab tests are ABNORMAL for Hemophilia A? (include wether they are prolonged or decreased)

A

PTT: Prolonged

Factor VIII: Decreased

(PT time is normal- no effect on extrinsic clotting)

41
Q

what is “Severe” hemophilia A? what is its characteristics?

A

<1% factor VIII

Recurrent spontaneous soft tissue and joint space bleeding

42
Q

what are the characteristics of moderate and mild Hemophilia A?

A

Moderate:
1 - 5% factor VIII
Bleeding with minor trauma

Minor:
>5% factor VIII
Bleeding with major trauma

43
Q

what treatments are used for Hemophilia A?

A

Factor VIII Concentrates

Fibrinolytic inhibitors

44
Q

list the disease related complications of Hemophilia A:

A

Arthritis and joint destruction
Pseudotumors
Intracranial bleeding
Retroperitoneal bleeding

45
Q

list the treatment related complications of Hemophilia A:

A

Antibodies to factor VIII

Infectious diseases

46
Q

list the characteristics of Hemophilia B:

A
  • Sex-linked, recessive disorder
  • Deficiency of factor IX (INTRINSIC pathway)
  • Clinical and laboratory presentation similar to deficiency of factor VIII

Mild, moderate, severe deficiencies are seen
Mild cases may be difficult to recognize clinically

47
Q

list the disease related complications of Hemophilia B:

A

SAME AS HEMOPHILIA A

Arthritis and joint destruction
Pseudotumors
Intracranial bleeding
Retroperitoneal bleeding

48
Q

list the treatment related complications of Hemophilia B:

A

Antibodies to factor IX

Infectious diseases

49
Q

what are the mechanisms for thrombocytopenia?

A

Decreased platelet production
Increased destruction
Sequestration
Congenital versus acquired

50
Q

list the clinical characteristics of Acute/childhood ITP

ITP = immune thrombocytopenia purpura

A 
Viral prodrome (early symptom) common
Sudden onset 
Severe thrombocytopenia
Frequently undergoes spontaneous remission
M:F close to 1:1
51
Q

list the clinical characteristics of Adult/chronic TTP

TTP = thrombotic thrombocytopenia purpura

A 
No antecedent infection
Gradual onset
Moderate thrombocytopenia
Infrequent spontaneous remission
More common in females
52
Q

what are the clinical characteristics of all ITP’s?

A

Petechiae
Ecchymoses (bruising)
Bleeding with trauma or surgery

53
Q

what is the pathophysiology for ITP?

A
  • Autoantibodies directed at the platelet membrane antigens
  • GPIb/IX and IIb/IIIa common targets
  • Increased IgG bound to the platelet surface promotes increased sequestration/destruction by the reticuloendothelial system
54
Q

what changes in the blood and bone marrow can be seen during ITP?

A

Megakaryocytes normal to increased

No microangiopathic changes on blood smear review

55
Q

Therapy for ITP includes:

A

Corticosteroids
Intravenous immunoglobulin
Immunosuppression
Splenectomy

56
Q

what is Thrombotic Thrombocytopenic Purpura (TTP)?

A
  • Acute disorder characterized by intravascular platelet activation with formation of platelet-rich microthrombi throughout the circulation
  • Left untreated, TTP is associated with high mortality.
57
Q

TTP is Now known to be caused by a deficiency of ____________, a metalloproteinase

A

ADAMTS 13

58
Q

what does the ADAMTS 13 metalloproteinase do?

A

degrades very-high-molecular-weight vWF

59
Q

what lab method is used to detect TTP?

A

HUS-TTP spectrum

60
Q

what is Disseminated Intravascular Coagulation (DIC) ?

A

Unregulated, widespread intravascular activation of the hemostatic system

Systemic formation of thrombin and plasmin with consumption of clotting factors and bleeding

61
Q

the result of DIC is the formation of what 2 conditions?

A
  • microvascular thrombosis

- hemorrhaging

62
Q

what can cause DIC?

A

Infections (gram negative sepsis)

Tissue injury (trauma, burn, surgery)

Obstetrical complications

Certain malignancies

63
Q

list all the clinical signs of DIC:

A 
Bleeding from multiple sites
Thromboembolic problems
Hypotension and shock
Respiratory dysfunction
Hepatic dysfunction
Renal dysfunction
CNS dysfunction
64
Q

During therapy for DIC, what blood products can be transfused?

A

FFP: coagulation & regulatory proteins

Cryo: fibrinogen, VIII, vWF

Platelets: platelets

65
Q

heparin works in the same way as what enzyme/Factor in the coagulation cascade?

A

antithrombin

gen-path 2 (7 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions