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Acquired Disorders Flashcards

1
Q

Factor V Leiden

Clinical Findings, Pathophysiology, Lab and therapy

A

Clinical finding: Caucasians; prone to thrombophilia as a result of venous thrombi

Pathophysiology: A mutation in the Factor V gene results in a conformation change in Factor V that makes its activated form resistant to Activated Protein C inhibition; Patients are unable to inhibit Factor Va

Lab: PT, PTT, plt count—normal
Diagnostic FVL PCR test

Therapy: antithrombotic treatment

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2
Q

Prothrombin 20210

Clinical Findings, Pathophysiology, Lab and therapy

A

Clinical Findings: Caucasians; At risk for Venous thrombosis; FVL mutation

Pathophysiology: A mutation in the prothrombin gene which leads to elevated prothrombin levels; Increased Factor II leads to thrombin formation which leads to excess thrombus formation

Lab: Prothrombin levels are high end of normal PCR is diagnostic

Therapy: antithrombotic agents

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3
Q

Protein C Deficiency

Clinical Findings, Pathophysiology, Lab and therapy

A

Clinical Findings: Recurrent DVT at a young age and PE
Homozygous—purpura fulminans, VT and DIC at birth
Heterozygous—skin necrosis within warfarin treatment

Pathophysiology: The diminishes capacity to destroy Factor Va and VIIIa results in an increased production of thrombin

Lab: PC Assay decreased

Therapy: warfarin; Liver transplant will “cure”

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4
Q

Protein S Deficiency

Clinical Findings, Pathophysiology, Lab and therapy

A

Clinical Findings: cases of arterial thrombi; warfarin induced skin necrosis possible

Pathophysiology: conditions where C4b-binding protein are increased; results in decreased functionality of Protein S to be able to act as a cofactor for Protein C, thus limiting the bodies capacity to inhibit factor Va and VIIIa, resulting in increased production of thrombin

Lab: PS Assays (total and free)

Therapy: warfarin, liver transplant

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5
Q 
Antithrombin Deficiency
Clinical Findings (Homozygote, Heterozygote), Pathophysiology (Type I and II), Lab and therapy
A

Clinical Findings: Recurrent venous thrombosis.
Homozygote: Type I: not compatible with life
Type II: severe thrombotic tendencies at birth
Heterozygote: asymptomatic when young; progress to more of DVT, PE with age

Pathophysiology: Type I—reduced synthesis; Type II—functional defect results in decreased normal inhibition of serine proteases of the coagulation cascade, leading to excess fibrin formation

Lab: AT assays

Therapy: Treatment with heparin and AT concentrates; Coumadin for long-term

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6
Q

Arterial thrombi

A

Formed in the arteries where blood flow is rapid; termed “white thrombi” because they are composed mostly of fibrin and platelets. Typically form at sites where endothelium is disturbed. Portions of the thrombus can break off (embolus) and lead to myocardial or cerebral infarction.

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7
Q

Venous thrombi

A

Formed in areas where blood flow is slow and disturbed. Composed mostly of RBCs and fibrin.

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8
Q

Antithrombin Deficiency Type I, II, III

Pathophysiology
Homozygotes
Heterozygotes

A

Pathophysiology: leads to inability to properly inhibit coagulation specifically serine proteases

Homozygotes: Type I: cannot survive, Type II: severe prenatal episodes
Heterozygotes: Symptomatic after puberty (DVT, PE, recurrence) Less severe in heparin binding site variant

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9
Q

Protein C Deficiency types

Homozygotes
Heterozygotes

Type I, II

A

Homozygotes: Thrombotic issues at birth (purpura fulminans)
Heterozygotes: Often asymptomatic until another risk factor presents: DVT, PE

Type I
Quantitative antigen quantity has decreased functional activity
Type II
Qualitative antigen quantity has normal functional activity

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10
Q

Protein S Deficiency types I, II, III

Pathophysiology

Types I, II, III

A

Pathophysiology: leads to inability to properly inhibit coagulation (specifically FVa and FVIIIa)

Type I
Quantitative Total protein: decreased
Free protein S: decreased
Protein S activity decreased

Type II
Qualitative Total protein normal
Free protein S: normal
Protein S Activity decreased

Type III
Increased C4bp and normal amounts of PS
Total protein S: normal 
Free protein S: decreased 
Protein S Activity: decreased
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11
Q

Deep Vein Thrombosis:

pathophysiology, lab testing, treatment.

A

Patho: Development of venous thrombosis in the deep veins of lower limbs. Symptoms include localized pain, warmth, redness, and swelling

Lab Testing: Thrombin generation and fibrinolysis are often elevated in cases of DVT, D-dimer

Treatment includes anticoagulant therapy, such as heparin

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12
Q

Pulmonary Embolism:

pathophysiology, lab testing, treatment

A

Patho: 50% of DVT cases lead to PE (termed venous thromboembolism—VTE), where a piece of the DVT breaks off and travels in circulation to the lungs, where it becomes lodged. Commonly fatal

Lab testing: venography or ultrasound confirmation

Treatment: anticoagulant therapy, such as heparin

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13
Q

Heparin Cofactor II Deficiency

A

Low thrombotic risk; decreased inhibition of thrombin, typically only a problem if another deficiency is present

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14
Q

Hyperhomocysteinemia

A

Mutation for enzyme required for HC breakdown (CBS or MTHFR) results in increased HC; increased HC is associated with premature atherosclerosis and thrombosis

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15
Q

Dysfibrinogenemia

A

Mutation that changes the structure of fibrinogen; decreases fibrinolytic activity because of 1) abnormal resistance to plasmin lysis or 2) reduced plasminogen activation

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16
Q

Elevated Factor VIII

A

Increased Factor VIII levels increased thrombotic risk because of increased thrombin formation and/or diminished APC effect

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17
Q

Factor XII Deficiency

A

Can be associated with thrombotic tendencies; thought to be the result because of Factor XII’s role in activation of fibrinolysis

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18
Q

Plasminogen Deficiency

A

The plasminogen deficiency can be either qualitative or quantitative. Less plasmin can be generated leading to decreased fibrinolytic capabilities

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19
Q

TTP (Thrombotic Thrombocytopenic Purpura)

A

vWF multimers cannot be properly cleaved; Ultralarge multimers directly agglutinate platelets causing thrombi

Deficiency in vWF cleaving proteases ADAMTS-13

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20
Q

HIT (Heparin induced Thrombocytopenia)

A

antibody/heparin/PF4 binds to platelets and leads to platelet activation and clearance

HIT: thrombocytopenia without thrombosis
HITT: thrombocytopenia with thrombosis

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21
Q

HUS (Hemolytic Uremic Syndrome)

A

Thought to be a result of endothelial damage from bacterial toxin (typically form E. coli O157:H7)

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22
Q

Malignancy

A

Thought to be the result of increased stasis, activation of blood coagulation and vascular injury

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23
Q

Pregnancy/Oral Contraceptives

A

Quantitative changes to hemostatic proteins lead to increased thrombotic risk, stasis, and also changes in hormone levels.

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24
Q

Discuss each item as it relates to the Lupus Anticoagulant definition

A

An antibody that reacts/binds with phospholipids; prolongs hemostasis screening tests that utilize a phospholipid-based reagent (PT and PTT)

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25
Discuss each item as it relates to the Lupus Anticoagulant. | Naming confusion
Originally found in patients with lupus but more common with patients without lupus, associated with autoimmune diseases, drugs, infections. Originally called anticoagulant because it prolonged screening tests like the PTT suggesting that it may have anticoagulant properties but later discovered that patient actually have thrombotic tendencies
26
Discuss each item as it relates to the Lupus Anticoagulant. | Clinical manifestation, application and pathophysiology
Clinical Manifestations & Applications Thrombotic in nature, increased risk for DVT, PE, arterial thrombosis, stroke Pathophysiology inhibition of endothelial cell anticoagulant processes and causing cells in contact with blood to acquire procoagulant phenotype; can interfere with protein C activation, inhibit heparin sulfate and prostacyclin release, and stimulate platelet aggregation
27
Discuss each item as it relates to the Lupus Anticoagulant. Lab results and Diagnosis protocol Implications in the Lab Diagnostic Protocol Confirmatory Procedures
Implications for the Laboratory: PTT prolonged cuz LA antibody reacts with the phospholipids in the reagent. PT occasionally prolonged Diagnostic Protocol Screening procedures—two or more using single concentration of phospholipids Demonstrate at least one prolonged phospholipid-based clotting test (PTT) Demonstrate at least one additional prolonged LA screening test (PT) Confirmatory procedures—modify abnormal screening procedure by altering phospholipid content of the test procedure, which demonstrates that the LA depends on phospholipid
28
Dilute Russell's Viper Venom Time (dRVVT) | Purpose/principle, Reagent, interpretation of results
Principle/Purpose: Also known as the Stypven Time; Reagent is added to patient plasma activating Factor and time to clot formation is measured. Reagent: Russell's viper venom, calcium chloride, and phospholipids Interpretation of results: Ratio of patient's clotting time to clotting time of normal control determined Normal ratio is <1:2 Increased ratio suggests the presence of LA
29
Plasma Clot time (PCT) for Lupus Anticoagulant | Purpose/principle, Reagent
Principle/Purpose: Reagent with a contact factor activator is added and the time to clot formation is measured (much like the PTT) Reagent: contact activator
30
Kaolin Clotting time (KCT) for Lupus Anticoagulant | Purpose/principle, Reagent, interpretation of results
Principle/Purpose: Kaolin is a substance that can activate contact factors; It is added to patient plasma and the time for the clot formation is measured Reagent: kaolin (a neg charged particulate activator) instead of phospholipid Interpretation of results: Test to control ratio of >1:2 indicates an inhibitor such as LA
31
Discuss normal and abnormal bleeding disorders that can arise in newborns. Include a description of clinical manifestations and pathophysiology.
Neonates are at risk for coagulation issues naturally The most common hemostatic problem is Vit K deficiency and immune thrombocytopenia
32
Discuss how each of the following lab tests could (or could not) be used to help differentiate liver disease and DIC. PT/PTT
Liver disease: PT, PTT, TT: increased because there is a lower amount of factors being produced DIC: PT, PTT, TT: increased because of the consumption of factors
33
Discuss how each of the following lab tests could (or could not) be used to help differentiate liver disease and DIC. Platelet count
Liver Disease: decreased because of enlarged liver → enlarged spleen→ more platelets sequestered DIC: decreased because of over activated and used up
34
Discuss how each of the following lab tests could (or could not) be used to help differentiate liver disease and DIC. D-dimer
Liver disease: normal: no clotting therefore no fibrinolysis DIC: Increased: excessive breakdown of the clots that were forming
35
Discuss how each of the following lab tests could (or could not) be used to help differentiate liver disease and DIC. Blood Smear
Liver Disease: Macrocytes, target cells, acanthocytes DIC: Schistocytes (RBC interacting with clots) and decreased platelets
36
Discuss how each of the following lab tests could (or could not) be used to help differentiate liver disease and DIC. Factor VIII assay
Liver Disease: Increased factor levels (acute phase reactant) DIC: decreased because used up
37
Discuss how each of the following lab tests could (or could not) be used to help differentiate liver disease and DIC. Factor IX assay
Liver Disease: decreased (production) DIC: decreased (used up)
38
Discuss how each of the following lab tests could (or could not) be used to help differentiate liver disease and DIC. Factor X assay
Liver Disease: decreased (production) DIC: decreased (used up)
39
Disseminated Intravascular Coagulation Clinical Manifestation and Pathophysiology
Clinical Manifestations Bleeding: abrupt, hemorrhage, multiple sites Pathophysiology Inappropriate activation of thrombin that leads to a systemic activation of coagulation that consumes factors and inhibitors needed for hemostatic control
40
Disseminated Intravasclar Coagulation Lab Results and Treatment
Expected Lab Results PT, PTT, TT elevated Low fibrinogen Platelet count decreased DD abnormal schistocytes RBCs are forced through fibrin clots, slicing them Treatment Options Eliminate the underlying cause; give RBCs, platelets, cryo or FFP DO NOT USE FIBRIN INHIBITORS
41
Liver Disease Clinical Manifestations and Pathophysiology
Clinical Manifestations Minimal bleeding except in severe disease states where ecchymoses and epistaxis are common Pathophysiology Liver is unable to keep up with its normal tasks of producing coagulation factors and removing activated factors Most hemostatic proteins are synthesized in the liver and liver macs play a major role in the removal of activated hemostatic components
42
Liver Disease Lab Results and Treatment
Expected Lab Results Platelet count decreased result of liver being enlarged→ enlarged spleen PT, PTT,TT increased lower amount of factors being produced Fibrinogen assay decreased lower amount of factors being produced DDimer Normal FDPs increased liver is unable to remove them in circulation Blood Morph acanth, target cells, macrocytes changes in RBC membrane lipids Treatment Options Replacement therapy as needed
43
Vitamin K Deficiency Clinical Manifestations and Pathophysiology
Clinical Manifestations Bleeding, HDN in newborns bleeding in the skin or from mucosal surfaces, circumcision Pathophysiology Not enough Vit K for Vit K dependent factors (II, VII, IX, X) to be properly synthesized, unable to participate in fibrin formation. Caused by dietary insufficiency, prolonged antibiotic therapy.
44
Vitamin K Deficiency Lab Results and Treatment
Expected Lab Results Platelet count normal PT, PTT increased II, VII, IX, X are all vitamin K deficient TT and Fibrinogen Assay Normal DDimer normal Protein C & S decreased Blood smear normal RBCs Treatment Options Administer Vitamin K, replacement of normal flora in GI tract
45
Primary Fibrinogenolysis Clinical Manifestations and Pathophysiology
Clinical Manifestations DIC like bleeding symptoms Pathophysiology Plasminogen becomes inappropriately activated to plasmin without thrombin generation
46
Primary Fibrinogenolysis Lab Results and Treatment
Expected Lab Results PT, PTT, TT elevated plasmin degrades fibrinogen and other factors Fibrinogen decreased Platelet count normal FDPs increased DDimer normal Treatment Options Epsilon aminocaproic acid (EACA)—a plasmin inhibitor
47
``` Oral Anticoagulants (ex. coumadin) Mode of action, reason for use, lab monitoring, results ```
Mode of action: Prevents liver from using Vit K therefore prevent carboxylation of factors so they can't bind to calcium and are rendered useless Reason for use: Long term treatment of DVT and PE—pill Lab monitoring: PT—increased INR—used to monitor oral anticoag dosing Results: Low/no oral anticoagulants: 1.0 Therapeutic Range: 2.0-4.0 Critical Value: 5.0
48
``` Oral Anticoagulants (ex. coumadin) potential complications, antidote, source ```
Potential complications Bleeding: increased dose Thrombosis: possible at start of the treatment, Vit K dependent factors (PS and PC) are inhibited before the rest therefore excess fibrin made and skin necrosis Antidote: Heparin and coumadin till PS and PC and other proteins are replaced Source:Vitamin K shot
49
Unfractionated Heparin | Mode of action, reason for use, lab monitoring,
Mode of action: Given intravenously because can't be absorbed via intestines Reason for use: To prevent further thrombosis; presurgical; to treat inpatients with DVT Lab monitoring: PTT—increased proportionally with dose Anti-Xa assay—new way of monitoring heparin
50
Unfractionated Heparin | potential complications, antidote, source
Potential complications: Bleeding—dose too high cuz to much fibrin inhibition Thrombocytopenia—heparin induced Osteoporosis and Resistance—long time use Antidote: Decrease dose or discontinue it cuz short half life Normal in few hours Source: Intestines of pigs or cow lungs
51
Explain why some hospital patients are receiving oral anticoagulants and heparin therapy simultaneously.
Heparin is given because it has an immediate effect. It is given intravenously so they must transition to oral anticoagulants so the hospital patients are on both of them since the oral anticoagulants take 3-5 days to take affect.
52
What is the INR? How is it calculated? How are the results interpreted (include a discussion of normal, therapeutic, and therapeutic ranges)?
Calculation: Patient PT results in seconds/mean of PT reference range in seconds) ISI: International Sensitivity Index—this number is assigned to thromboplastin reagent the closer it is to 1.0 the purer it is Results Normal (around 1.0)—patient not on oral anticoagulants Therapeutic (2.0-4.0) Critical (>5.0) Dose of oral anticoagulants too high
53
Low Molecular Weight Heparin | Mode of action, indications for use, lab monitoring, potential complications
Mode of Action: Catalyzes the interaction of Antithrombin with Factor Xa, leading to increased inhibition of thrombin and other serine protease Indications for Use: An alteration to unfractionated heparin for patients with thrombosis to treat current condition Lab Monitoring: PTT not recommended; anti-Xa assay preferred Potential Complications: Rare; reduced occurrence of HIT (compared to UFH) and fewer adverse complication
54
Thrombolytic therapy | Mode of action, indications for use, lab monitoring, potential complications, examples
Mode of Action: Plasminogen activators used to lyse thrombi in vivo Indications for Use: Myocardial infarction, acute stroke, PE, DVT, peripheral arterial occlusion Lab Monitoring: TT is prolonged and can be used to monitor therapy; FDPs and DDimer would be elevated if therapy is working Potential Complications: Lysis of normal fibrin clots can lead to bleeding Examples: Streptokinase, anything with kinase
55
Antiplatelet therapy | Mode of action, indications for use, lab monitoring, potential complications, examples
Mode of Action:Inhibits platelet function Indications for Use: Current or at-risk arterial thrombosis patients; in dental implant procedures Lab Monitoring: Platelet count might be monitored to watch for thrombocytopenia caused by certain drugs; Platelet aggregation and PFT tests would be abnormal Potential Complications: Platelet like bleeding (petechiae, oozing), Thrombocytopenia Examples: COX-1 inhibitors (Asprin), ADP receptor inhibitors (ex: Plavix), phosphodiesterase inhibitors; GPIIb/IIIa inhibitors
56
What effect do thrombolytic agents have on lab results?
Increased: PT, PTT, TT, FDP, Plasmin Decreased: Fibrinogen, Plasminogen, α2-antiplasmin
57
Discuss laboratory tests used in the evaluation of hypercoagulable states: Antithrombin III
Chromogenic is a coupled enzymatic reaction with thrombin in the presence of heparin. Thrombin activity is inversely proportional to the amount of AT-III in plasma, p-nitroaniline is measured at 405nm. Normal 85-122%
58
Discuss laboratory tests used in the evaluation of hypercoagulable states: Protein C
Deficiencies associated with Vitamin K deficiency, liver disease, DIC and oral anticoagulant therapy Measures p-nitroaniline, amount directly proportional to amount of activated protein C.
59
Discuss laboratory tests used in the evaluation of hypercoagulable states: Protein S
Measures the free protein S fraction. Clotting time prolonged in the presence of increased levels of protein S. Associated disease states include liver disease, diabetes mellitus, pregnancy, DIC, and oral anticoagulant therapy
60
Discuss laboratory tests used in the evaluation of hypercoagulable states: Plasminogen
Coupled enzymatic reaction, utilizing streptokinase. Measures activity of p-nitroaniline. Useful in determining effectiveness of plasminogen therapy
61
Discuss laboratory tests used in the evaluation of hypercoagulable states: Antiplasmin
Measures circulating α2-antiplasmin, useful in determining efficacy of fibrinolytic therapy. Normal levels 80-120%
62
Discuss laboratory tests used in the evaluation of hypercoagulable states: D-dimer
Detects unique breakdown products of fibrin; useful as a screen/negative predictor of DVT
63
Electromechanical Methodology
Measures the time for clot formation by detecting changes in the reaction mixture Fibrometer detects the completion of an electrical circuit that occurs when fibrin forms between two electrodes
64
Optical Density Methodology
Detects changes in optical density that occur when fibrin forms
65
Chromogenic Methodology
An enzyme (such as an activated coagulation factor) cleaves a chromogenic substrate that releases a chromophore tag; color intensity is measured spectrophotometrically and is directly proportional to the concentration of the chromophore tag.
66
Antithrombin Deficiency Type I, II, III
Type I Quantitative Concentration: decreased Heparin cofactor: decreased Progressive AT: decreased Type II (Active Site Defect) Qualitative Concentration: normal Heparin Cofactor: decreased Progressive AT: decreased Type II (Heparin-binding Site Defect) Qualitative Concentration: Normal Heparin Cofactor: decreased Progressive AT: normal

Hemostasis (3 decks)

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