Hemostasis: Pathoma Flashcards

1
Q

What is hemostasis

A

Repair to damaged blood vessels through formation of a clot/thrombus

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2
Q

Hemostasis occurs in what stages?

A

Primary

Secondary

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3
Q

Primary hemostasis forms what?

What mediates this?

A

Weak platelet plug

Interaction between platelets and vessel wall

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4
Q

Secondary hemostasis has what effect?

What mediates?

A

Platelet plug

Coagulation cascade

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5
Q

4 steps of primary hemostasis

A
  1. Transient vasoconstriction of BV
  2. Platelet adhesion
  3. Platelet degranulation
  4. Platelet aggregation
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6
Q

What mediates transient vasoconstriction of BV?

A

Reflex neural stimulation

Endotheli release from endothelial cells

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7
Q

How do platelets adhere to surface of damaged BV? 2

A
  1. vWF binds to exposed collagen

2. Platelets bind to vWF using GPIb receptor

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8
Q

vWF is derived from what two locations?

A
  1. Weibel-Palade bodies of endothelial cells

2. Alpha granules of platelets

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9
Q

In platelet degranulation, what is released? 2

A
  1. ADP from platelet dense granules

2. TXA2 from platelet COX enzyme

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10
Q

ADP has what effect?

A

Promotes exposure of GPIIb/IIIa receptor on platelets

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11
Q

TXA2 has what funciton?

A

Promotes platelet aggregation

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12
Q

Platelet aggregation occurs how?

A

Platelets aggregate at site of injury via GPIIb/IIIa using fibrinogen as a linking molecule

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13
Q

Disorders of primary hemostasis are due to what?

A

Qualitative or quantitative changes to platelets

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14
Q

2 sets of clinical features of primary hemostasis

A

Mucosal bleeding

Skin bleeding

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15
Q

Most common skin bleeding symptom in primary hemostasis disorder?

A

Epistaxis

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16
Q

Most feared complication of mucosal bleeding?

A

Intracranial bleeding

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17
Q

4 useful platelet counts in determining disorders?

A
  1. Platelet count
  2. Bleeding time
  3. Blood smear
  4. Bone marrow biopsy
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18
Q

Immune thrombocytopenic purpura definition

A

Autoimmune production of IgG against platelet antigens

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19
Q

Who makes the antibodies in ITP?

What eats the antibody-bound platelets?

A

Spleen

Spleen

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20
Q

Acute form of TPP is in what age?

A

Kids

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21
Q

Chronic ITP is in what age?

A

Adults

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22
Q

What happens if pregnant woman gets ITP?

A

Fetus will get some anti-platelet Ab’s that will cross placenta and have effects for awhile.

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23
Q

Lab findings in ITP? 3

A
  1. Decrease platelet count
  2. normal PT/PTT
  3. Increase in megakaryocytes in BM biopsy
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24
Q

First Treatment of ITP?

A

Corticosteroids

25
Secondary treatment of ITP?
1. IVIG to raise platelet count | 2. Splenectomy to remove source of Ab's and platelet destruction
26
Microangiopathic hemolytic anemia results in formation of what?
Platelet microthrombi in small vessels
27
MHA has what effect on platelets and RBC's
Consumes platelets RBC's are sheared resulting in hemolytic anemia with schistocytes
28
Two causes of MHA?
Thrombotic thrombocytopenic purpura Hemolytic Uremic syndrome
29
TTP is due to what?
Auto Antibodies against the enzyme ADAMS13 which results in large polymers of vWF causing abnormal platelet adhesion and microthrombi
30
TTP common population?
Adult females
31
HUS is due to what? Main example
Endothelial damage by drugs or infection causing platelet microthrombi to form E. coli O157:H7 verotoxin
32
Clinical findings of both MHA's?
1. Skiin and mucosal bleeding 2. Hemolytic anemia 3. Fever 4. Renal insufficiency (HUS) 5. CNS problems (tTP)
33
Lab findings in both MHA's? 5
1. Thrombocytopenia 2. Increased bleeding time 3. Normal PT/PTT 4. Anemia with schistocytes 5. Increase in megakaryocytes
34
Treatments of hemostasis? 2
Plasmapheresis and corticosteroids
35
Bernard Soulier syndrome is a genetic deficiency in what? | Result?
GPIb deficiency Platelet adhesion impaired
36
Glanzmann thrombasthenia is due to what? What is result
Genetic GPIIb/IIIa deficiency Platelet aggregation impaired
37
Aspirin's effect on platelets?
Inhibits COX --> lack of TXA2 --> Impaired aggregation
38
Uremia effect on platelets?
Impairs platelet function --> Both adhesion and aggregation impaired
39
Purpose of secondary hemostasis?
Stabilize weak plug via the cogulation cascade
40
End product of coagulation cascade?
Thrombin
41
What does thrombin do?
Converts fibrinogen in the platelet plug to fibrin.
42
Function of fibrin?
Cross-link and yield a stable platelet fibrin thrombus
43
Coagulation factors are produced where?
Liver
44
Activation of coagulation factors requires what? (3)
1. Exposure to an activating substance 2. Phospholipid surface of platelets 3. Calcium
45
Disorders of secondary hemostasis is usually due to what?
Factor abnormalities
46
Clinical features of secondary hemostasis?
Deep tissue bleeding into muscles and joints | Rebleeding after surgical procedures
47
What is goal of both coag pathways?
Make 10
48
Intrinsic factor pathway?
12, 11, 9, 8, 10
49
Extrinsic factor pathway?
Just 7
50
What activates Factor 12 in intrinsic?
Subendothelial collagen
51
What activates Factor 7 in extrinsic?
Tissue thromboplastin
52
PT measures what?
Extrinsic pathway (factor VII)
53
PTT measures what?
Intrinsic pathway (factors XII, XI, IX, VIII)
54
PTT measures what drug?
Heparin
55
PT measures what drug?
Coumadin
56
Hemophilia A is defect in what? Heredity? Can you acquire the mutation?
Factor 8 deficiency X-linked recessive` Yes
57
Lab findings in hemophilia A? PTT/PT Blood serum Platelet count and bleeding time
High PTT, normal PT Decrease factor 8 in blood Normal platelet count and bleeding time
58
Treatment of hemophilia A?
recombinant factor 8
59
Hemophilia B is also known as? Deficiency of what? Difference from hemophilia A?
Christmas disease Factor 9 Factor 9 levels are decreased