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Hemostasis and Related Disorders Flashcards

1
Q

End product of primary hemostasis

A

Platelet plug

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2
Q

End product of secondary hemostasis

A

Stabilize the weak platelet plug

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3
Q

Immediate response to endothelial damage

A

Transient vasoconstriction

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4
Q

Function of von Willebrand Factor

A

Binds to exposed collagen in damaged endothelium and the platelets then bind to the vWF

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5
Q

Platelets bind to vWF via what receptor

A

Gp1b

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6
Q

Source of von Willebrand factor

A 
Endothelial cell (majority)
platelet
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7
Q

Contents and location of Weibel palade body?

A

vWF and P selectin

found on endothelial cell

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8
Q

Binding of platelets to vWF causes degranulation which releases what substances and function

A 
ADP - causes expression of GpIIbIIIa necessary for aggregation
Thromboxane A2 (from platelet COX) - promotes platelet aggregation
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9
Q

Molecule which links platelets together

A

Fibrinogen

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10
Q

Thrombocytopenia usually presents with?

A

Mucosal bleeding (hemoptysis, skin, GI, hematuria) (intracranial if severe)

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11
Q

Autoimmune production of IgG against platelet antigens (like GpIIbIIIa)

A

Immune Thrombocytopenic Purpura

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12
Q

Most common cause of thrombocytopenia in children and adults

A

Immune Thrombocytopenic Purpura

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13
Q

Where are autoantibodies against platelets produced in ITP

A

Spleen

Spleen then destroyes these antibody tagged platelets

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14
Q

Lab findings in ITP

A

low platelet count
normal pt/ptt
increased megakaryocytes (compensation)

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15
Q

Initial Treatment for ITP

A

Corticosteroids, IVIG

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16
Q

How does IVIg work

A

Introduce immunoglobulin to body, spleen destroys that immunoglobulin instead of the autoimmune process

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17
Q

Pathologic formation of platelet microthrombi in small vessels

A

Microangiopathic Hemolytic Anemia

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18
Q

Pathophysiology of hemolytic anemia in Microangiopathic Hemolytic Anemia

A

If there is microthrombus, RBC passing through narrow vessel is damaged by the thrombus, yielding schistocytes.

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19
Q

What is a schistocyte

A

Damaged RBC, characteristic of hemolytic anemia

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20
Q

Two types of Microangiopathic Hemolytic Anemia and difference bet two

A

Thrombotic Thrombocytopenic Purpura - Hemolytic Uremic Syndrome

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21
Q

Enzyme decreased in TTP and function

A

ADAMSTS13 - degrades (prepares) vWF polymers to monomers for proper platelet adhesion,

if polymers remain, platelet excessively aggregates –> thrombus

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22
Q

Pathophysiology of Hemolytic Uremic Syndrome

A

Drugs or infection damage endothelium –> microthrombi –> shearing of RBC

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23
Q

Infection associated with HUS

A

E coli O 157:H7 - releases verotoxin

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24
Q

Clinical findings of TTP and HUS

A

Both - fever, hemolytic anemia, thrombocytopenia
TTP - CNS abnormality
HUS - Renal insufficiency

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25
Treatment of TTP or HUS
Plasmapheresis, corticosteriois
26
Genetic Gp1b deficiency, leading to impaired platelet adhesion
Bernard Soulier Syndrome
27
Blood smear finding in Bernard Soulier syndrome?
Mild thrombocytopenia, enlarged platelets (immature, short lived platelets)
28
Genetic GpIIbIIIa deficiency
Glanzmann thrombastenia
29
End product of secondary hemostasis
Generation of thrombin which turns fibrinogen to fibrin, and fibrin is cross linked forming stable platelet plug
30
Clinical features of disorders of clotting factors
Deep tissue bleeding (muscles and joints), rebleeding after surgery
31
PT vs PTT
PT - extrinsic and common pathway | PTT - intrinsic and common pathway
32
Intrinsic, Extrinsic, and common pathwayas (factors only)
Intrinsic - 12 - 11 - 9 - 8 - 10 Extrinsic - 7 - 10 Common - 5 - 2 - 1
33
What activates the intrinsic pathway?
Subendothelial collagen
34
What activates the extrinsic pathway?
Tissue thromboplastin
35
Heparin improves which clotting time?
PTT
36
Coumadin improves which clotting time?
PT
37
Disease with factor 8 deficiency, x linked recessive but can arise de novo from mutation
Hemophilia A
38
Disease with factor 9 deficiency
Hemophilia B, Christmas Disease
39
Acquired antibody against clotting factors? What is most common?
Clotting factor inhibitor | Anti - Factor 8
40
Utility of mixing study and logic behind it
Mix normal plasma with patient plasma If hemophilia - PTT improves (clotting factors of normal plams) IF clotting factor inhibitor - PTT still deranged (antibody attacks new cloting factors)
41
Most common inherited coagulation disorder
Von Willebrand disease (vWF deficiency)
42
Ristocetin test is used for what disease
Von Willebrand disease
43
Von Willebrand factor helps in stabilizing what clotting factor
Factor 8
44
Treatment for von willebrand disease
Desmopressin (*increase vWF release from weibel palade bodies)
45
Vitamin K does which step in the Vit K dependent factors
Gamma carboxylation
46
What enzyme activates Vitamin K and in what organ
Epoxide reductase - liver
47
Pathology of heparin induced thrombocytopenia
Heparin binds to platelet factor 4, which leads to IgG mediated platelet destruction, , then fragments activate other platelets leading to thrombosis
48
Pathologic activation of coagulation cascade
Disseminated Intravascular coagulation
49
Clinical features of DIC
Ischemia and infarction from coagultion | Mucosal bleeding from consumption of platelets and factors
50
What reptile when bites can cause DIC
Rattlesnake
51
Product from splitting of cross linked fibrin during fibrinolysis
D-dimer This is why used for screening DIC, pulmo embo
52
What converts plasminogen to plasmin
tissue Plasminogen Activator
53
Functions of plasmin
1. breaks fibrin 2. breaks fibrinogen 3. breaks clotting factors 4. prevents platelet aggregation
54
What molecule inactivates plasmin? | Where is it produced?
Alpha 2 anti-plasmin | Liver
55
This blocks activation of plasminogen, which can be used for treating disorders of fibrinolysis
Aminocaproic acid
56
Features of a thrombus which differentiates it from postmortem clot
1. Lines of Zahn | 2. Attached to vessel wall
57
What are Lines of Zahn
Layer of alternating blood cells and fibrin found in thrombosis
58
What is Virchow's Triad
Hypercoagulable state Stasis/Turbulence Endothelial Cell Damage
59
Deficiency of which vitamins can lead to homocystenuria?
Folate and B12
60
Characterized by vessel thrombosis,mental retardation long slender fingers, and lens dislocation from elevated homocysteine levels
Cystathionine beta synthase deficiency
61
Protein C and Protein S function?
Inactivate Factor 5 and 8
62
Pathophysiology of Warfarin skin necrosis
Protein c AND s are destroyed before vitamin K is, so less anticoagulant means more thrombosis in the skin, hence name
63
What is Factor V Leiden
Mutated form of Factor V with no cleavage site for protein C and S
64
Most common inherited cause of hypercoagulable state
Factor V Leiden
65
Inherited point mutation in prothrombin
Prothrombin 20210A
66
Decreased protective effect of heparin like molecules on endothelium
Antithrombin III deficiency | antithrombin prevents excessive clotting
67
Effect of estrogen on coagulation factors
Increased coagulation factor production --> increased thrombosis
68
Feature of atherosclerotic embolus
Cholesterol clefts in embolus
69
Bends and Chokes are associated with?
``` Decompression sickness (Nitrogen embolism) Muscle and joint pain; respiratory distress ```
70
Chronic form of decompression sickness associated with multifocal ischemic necrosis of bone
Caisson disease
71
Sustance in amniotic fluid which triggers coagulation cascade and may lead to DIC
Tissue Thromboplastin
72
Histologic findings in amniotic fluid embolus
Squamous cells and keratin debris
73
Gross appearance of pulmonary infarction
Hemorrhagic wedge shaped infarct
74
Complication of reorganization of chronic pulmonary embolus
Pulmonary Hypertension

PLE Pathology (10 decks)

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