Exam 1 I am tired and sad and running out of hope Flashcards

(79 cards)

1
Q

Define Hemostasis

A

Stopping blood flow with a fine balance between bleeding and clotting

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2
Q

What parameters are involved in hemostasis?

A

Vacular, platelets, fibrin clot formation - coagulation cascade, control (inhibitory systems), fibrinolysis

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3
Q

Procoagulant - what are things that participate in coagulation?

A

Platelets, vWF, coagulation factors, collagen

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4
Q

What does a deficiency in procoagulants result in?

A

BLEEDING
(except XII, Ia, PK, HMWK dysfunction)

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5
Q

Anticoagulant - what are things that participate in the regulation or inhibition of coagulation?

A

Antithrombin, Protein C, Protein S, HC II, Protein Z/ZPI, TFPI

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6
Q

What is the function of antithrombin?

A

Inactivates IIa, Xa, (IXa, XIa, XIIa, plasmin)

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7
Q

What is the function of Protein C?

A

Inactivates thrombin and thrombomodulin

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8
Q

What activates Protein C?

A

Thrombin and thrombomodulin

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9
Q

What is the function of Protein S?

A

In its free form, it is a Protein C cofactor

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10
Q

What is the function of HCII?

A

It neutralizes IIa (thrombin)

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11
Q

What is the function of TFPI?

A

Inhibits TF3/VIIa complex

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12
Q

What does a deficiency in anticoagulants result in?

A

THROMBOSIS

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13
Q

Profibrinolytic - what are things that participate in fibrinolysis?

A

Plasminogen (to plasmin), tPA, Factor XIIa, Kallikren, Urokinase

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14
Q

What is the function of Plasminogen (to plasmin)?

A

Lyses fibrin and fibrinogen and inactivates Va, VIIIa, and XIIa

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15
Q

What is the function of tPA?

A

Catalyzes the conversion of plasminogen to plasmin

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16
Q

What is the function of kallikren?

A

Formation and release of bradykinin

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17
Q

What is the function of urokinase?

A

Enzyme that converts plasminogen to plasmin

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18
Q

What does a deficiency in profibrinolytics result in?

A

THROMBOSIS

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19
Q

Antifibrinolytic - what are things that participate in the regulation or inhibition of fibrinolysis?

A

PAI-1 & 2, Alpha-2 Antiplasmin, Alpha-2 Macroglobulin, TAFI, Factor XIa

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20
Q

What is the function of PAI-1 & 2?

A

Neutralize tPA

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21
Q

What is the function of Alpha-2 Antiplasmin?

A

Binds plasmin

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22
Q

What is the function of Alpha-2 Macroglobulin?

A

Binds plasmin

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23
Q

What is the function of TAFI?

A

Alters the fibrin clot so that it is less recognizable by plasmin

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24
Q

What is the function of XIa?

A

Enhances TAFI production

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25
What does a deficiency in antifibrinolytics result in?
BLEEDING
26
What is the difference between FDP and D-Dimer?
D-Dimer is a specific FDP only present upon the degradation of a cross linked fibrin. Factor 13 has stabilized the clot
27
FDP stands for what? What are FDPs?
Fibrinogen Degradation Products Fragments X, Y, D, and E are FDPs
28
What are primary fibrinolysis intermediates?
X and Y
29
What are primary fibrinolysis end products?
D and E
30
T/F: FDPs can act as coagulation inhibitors by attaching to fibrin monomer and preventing polymerization and clot formation
TRUE
31
Function of anticoagulants?
Inhibit the coagulation cascade
32
What anticoagulants are we concerned with?
Coumadin/Warfarin Heparin Direct Thrombin Inhibitors Direct Xa inhibitors
33
Mechanism of Coumadin/Warfarin
Keeps Vitamin K in storage form Stops Vitamin K dependent factors (II, VII, IX, and X)
34
Mechanism of Heparin
Increase inhibitory effect of AT which creates an irreversible complex with IIa, IXa, Xa, XIa, XIIa, and plasmin
35
Mechanism of Direct Thrombin Inhibitors
Neutralizes thrombin by binding to the active site and extending fibrin bound thrombin
36
Mechanism of Direct Xa inhibitors
Inhibits Factor Xa (no AT requires)
37
Function of Anti-Platelets
Prevents platelet aggregation
38
What drug that we are concerned with is an anti-platelet?
Aspirin
39
Mechanism of Aspirin
Inhibits cyclooxygenase to reduce TXA2 production (which stimulates activation of new platelets and increases aggregation)
40
Mechanism of thrombolytics (Fibrin specific or fibrin non-specific)
Dissolves the clot
41
What is the type of disorder, defect, and laboratory results we would see in Von Willebrands (Type 1)
Disorder: Adhesion Defect: vWF deficiency (all multimers) Tests: PFA = INC PT = normal APTT = INC (d/t DEC VIII_ PLT Aggregation = abnormal Ristocetin (will correct with addition of NP)
42
What is the type of disorder, defect, and laboratory results we would see in Bernard-Soulier
Disorder: Adhesion Defect: Lack of Gp1b Tests: PFA = INC PLT = DEC Large PLT PLT Aggregation = abnormal Ristocetin
43
What is the type of disorder, defect, and laboratory results we would see in Glanzmann's
Disorder: Aggregation Defect: Lack GPIIb and GPIIIa Tests: PFA = INC PT = normal APTT = normal PLT count = normal PLT aggregation = normal ONLY w/Ristocetin
44
What is the type of disorder, defect, and laboratory results we would see in Delta Storage Pool
Disorder: Release Defect: Lack of dense granules Tests: Hypo or granular plts
45
What is the type of disorder, defect, and laboratory results we would see in Gray Platelet
Disorder: Release Defect: Marked DEC in platelet alpha granules Tests: Hypo or granular plts
46
What is the type of disorder, defect, and laboratory results we would see in ITP
Disorder: Thrombocytopenia Defect: Spontaneous -- adults/AIDS; kids/immunization Tests: PFA = INC PLT count = LOW <20,000 Large plt (variable) BM = megakaryocyte hyperplasia Deficient clot retraction
47
What is the type of disorder, defect, and laboratory results we would see in TTP
Disorder: Thrombocytopenia Defect: Autoantibody against ADAMTS13, vWF cannot break apart Tests: - Hemolytic anemia with schistocytes - Thrombocytopenia/hemorrhage - Fluctuating neurological dysfunction - Fever - Progressive renal disease
48
What is the type of disorder, defect, and laboratory results we would see in HUS
Disorder: Thrombocytopenia Defect: Resembles TTP with NO neurologic symptoms. Plts are sequestered in kidney Tests: - MAHA - thrombocytopenia - acute renal failure (RBCs, protein, and casts in urine)
49
What is the type of disorder, defect, and laboratory results we would see in HELLP
Disorder: Thrombocytopenia Defect: Release of TXA2. Microvascular endothelial damage. Vascular lesions. Intravascular plt activation Tests: - Hemolysis: hgb/hct, LDH, bili, Hapt, schisto - PLT count = LOW <200,000 - Hepatic dysfunction (AST >42, ALT >24, LDH >164)
50
What is the type of disorder, defect, and laboratory results we would see in drug induced thrombocytopenia
Disorder: thrombocytopenia Defect: Antibody complex attaches to platelet Tests: - Plt count LOW - Test for the presence of heparin-induced ab in the patient plasma (use a plt aggregometer; if ab is present, plts will aggregate)
51
What is the site of formation of the coagulation factors?
Liver
52
List the Vitamin K dependent factors
II, VII, IX, and X
53
Factor I
Fibrinogen Clot formation
54
Factor II
Prothrombin Activation of factors I, V, VII, VIII, XI, XIII, Protein C, and platelets
55
Factor III
Tissue factor III Cofactor of VIIa
56
Factor IV
Calcium Role in binding of phospholipid coagulation factors
57
Factor V
Labile Factor Cofactor of X -- prothrombinase complex
58
Factor VII
Proconvertin Enables factors IX and X
59
Factor VIII
von Willebrand Factor Complex Cofactor of IX complex
60
Factor IX
Christmas Factor Forms the complex with VIII; Enables factor X
61
Factor X
Stuart-Prower Factor Forms the prothrombinase complex together with Factor V, which will activate Factor II
62
Factor XI
Activates factor IX
63
Factor XII
Hageman Factor Enables factors XI, VII, and Prekallikrein
64
Factor XIII
Fibrin Stabilizing Factor Creates cross-links between fibrin monomers
65
How does Waldenstrom's macroglobulinemia and multiple myeloma can cause bleeding tendencies?
Igs coating platelets and collagen fibers
66
How does the following vascular disorder effect coagulation: hereditary hemorrhagic telangiectasia
- Vessel walls are reduced to single layer of EC and inadequate support structures resulting in fragile vessels - Telangiectasias: Dilated superficial vessels that will blanche (lose color with pressure, unlike petechiae - Bleeding
67
How does the following vascular disorder effect coagulation: Ehlers-Danlos Syndrome
- Indian rubber man (hypermobile joints and stretchy skin) - Collagen disorder: Issues with adhesion
68
How does the following vascular disorder effect coagulation: Allergic and drug-induced purpura
- Autoimmune vacular injury - Drug causes development of antibody to vessel walls
69
How does the following vascular disorder effect coagulation: Henoch-schonlein purpura
- Usually a child following upper respiratory infection - IgA deposits in vessels: Rash, abdominal pain, joint pain, proteinuria/hematuria. Small % advance to renal disease
70
How does the following vascular disorder effect coagulation: Scurvy
- Vitamin C deficiency - Decreased synthesis of collagen results in weakened capillary walls
71
How does the following vascular disorder effect coagulation: Senile Purpura
- Elderly - Usually forearms and back of hands - Loss of collagen and subcutaneous fat/elastic fibers to support for small blood vessels
72
Common agonist or aggregating agent of von willebrands
Ristocetin - corrects with NP
73
Common agonist or aggregating agent of bernard soulier
Ristocetin
74
Common agonist or aggregating agent of Glanzmann's Thrombasthenia
All abnormal except ristocetin
75
Common agonist or aggregating agent of Delta storage pool disease
ADP
76
Common agonist or aggregating agent of Gray platelet
ADP
77
Common agonist or aggregating agent of Aspirin or NSAID therapy
Arachidonic acid curve = more sensitive
78
von Willebrand factor activity– Ristocetin co-factor (vWF: RCo) -- Ristocetin Induced Platelet Agglutination
Uses patient PRP - patient's own platelets and plasma Normal with all agents except Ristocetin Add NP and it will correct for VWD
79
von Willebrand factor activity– Ristocetin co-factor (vWF: RCo) -- Ristocetin Cofactor (vWF:Rco)
Uses patient's plasma and donor or lyophilized platelets Measures the ability of patient's plasma cWF to agglutinate donor platelets in the presence of Ristocetin