Coagulation Disorders

Question 1

Thromboplastin aka

Answer 1

Tissue Factor

Question 2

Mechanism of Warfarin

Answer 2

Blocks epoxide reductase enzyme in liver, so Vitamin K cannot be reduced, thereby inhibiting coagulation by decreasing production of Factors II, VII, IX, and X that require reduced Vitamin K in order to work

Question 3

How long does it take normal person to form fibrin clot?

Answer 3

Intrinsic pathway- 28-30 seconds

Extrinsic pathway- 9-12 seconds

Question 4

Factor IX aka

Answer 4

Christmas factor

Question 5

What are the hereditary bleeding coagulation disorders

Answer 5

hemophilias, afibrinogenemia, clotting factor deficiencies

Question 6

How is hemophilia A inherited?

Answer 6

X-linked in 70% of cases, factor VIII deficiency, 30% are spontaneous mutations

Question 7

How is hemophilia B inherited?

Answer 7

X-linked, one third have abnormalities in how Factor IX works, 2/3rds have deficiency

Question 8

Manifestation if 5-75% of normal Factor VIII level

Answer 8

mild bleeding

Question 9

Manifestation if 1-5% of normal Factor VIII level

Answer 9

moderate bleeding

Question 10

Manifestation if less than 1% of normal Factor VIII level

Answer 10

Severe bleeding

Question 11

Hemophilia A and B clinical findings

Answer 11

Easy bruising, spontaneous bleeding, hemorrhages, and hemarthrosis

Question 12

1 unit of Factor VIII

Answer 12

equal to 100ng in 1 ml of plasma

Question 13

How do you raise levels of Factor VIII by 2%?

Answer 13

Give them 1 unit of Factor VIII/kg. So if they are 70 kg, have to give them 70 units

Question 14

Complication of giving Factor VIII concentrates

Answer 14

Too much can cause antibody formation and cause inhibition of Factor VIII

Question 15

Tx options for hemophilia A

Answer 15

Cryoprecipitate, FFP- restock on clotting factors that you are deficient in. DDAVP, EACA, Factor VIII concentrates

Question 16

DDAVP mechanism

Answer 16

Stimulates endothelial cells to release more Vwf from its granules, causing increased vwf and therefore increasing the half-life of Factor VIII. Tx for VonWillebrand Disease and Hemophilia A

Question 17

EACA

Answer 17

Epsilon Alpha Caproic Assay, antifibrinolytic medication given in tx of Hemophilia A and C- binds to plasmin and inactivates it to stop fibrin clot breakdown

Question 18

Benefits of prophylaxis of Factor VIII concentrates in hemophilia A

Answer 18

Reduces detectable joint abnormalities

Question 19

Which factor deficiency is not really associated with bleeding problems?

Answer 19

Factor XII deficiency- only 8-10% have a bleeding problem

Question 20

Hemophilia most common in Ashkenazi jews

Answer 20

C

Question 21

Tx for afibrinogenemia

Answer 21

cryoprecipitate

Question 22

which hemophilia causes mild bleeding post-op?

Answer 22

Hemophilia C

Question 23

The only coagulation bleeding disorder that has elevated levels of FDP or d-dimers:

Answer 23

DIC

Question 24

Diagnosis of DIC

Answer 24

Hypofibrinogenemia, elevated FDP levels, prolonged PT (and PTT), and thrombocytopenia

Question 25

Causes of DIC

Answer 25

Sepsis is most common cause, OB complications, tissue injury, cancer

Question 26

Tx of DIC

Answer 26

FFP, cryoprecipitate, platelets, and factors. TREAT UNDERLYING CAUSE (sepsis- empiric antibiotics), ensure organs are being perfused

Question 27

Acquired causes of coagulation bleeding disorders

Answer 27

Vitamin K deficiency, Liver disease, DIC

Question 28

What effects would liver disease have on clotting?

Answer 28

Vitamin K cannot be reduced, so coag factors cannot be produced from liver, increased fibrinolysis because alpha 2-antiplasmin synthesized in liver and can no longer bind to plasmin to inhibit clot breakdown- so too much breakdown--increased bleeding

Question 29

Tx for liver disease to replace clotting factors

Answer 29

FFP or cryoprecipitate if fibrinogen is less than 80mg/dl

Question 30

Patient on chronic antibiotics for the lat 10 years. Also has Chrome's disease and is complaining of increased diarrhea. What are you worried about?

Answer 30

Vitamin K deficiency- abx use and GI issues are wiping out intestinal bacteria which is one of the 2 sources for Vitamin K (besides leafy vegetables)

Question 31

What is the major culprit in causing DIC

Answer 31

Uncontrolled thrombin generation leading to small small clots in microcirculation

Question 32

Inherited coag disorders resulting in too much clotting

Answer 32

Factor V Leiden, Antithrombin III deficiency, Protein C deficiency, Protein S deficiency

Question 33

How is antithrombin III deficiency inherited?

Answer 33

Autosomal dominant

Question 34

10% of Dutch people and 5% of normal population have this disorder

Answer 34

Factor V Leiden

Question 35

How is factor V leiden inherited?

Answer 35

Can be homozygous or heterozygous- mutation in factor V gene

Question 36

How is protein C deficiency inherited

Answer 36

autosomal dominant

Question 37

2 types of protein C deficiency

Answer 37

Type I- quantitiative | Type II- qualitative

Question 38

antiphospholipid syndromes characterized by

Answer 38

anticardiolipin antibodies or lupus anticoagulants

Question 39

Non-inherited causes of hypercoagulable states leading to increased clotting

Answer 39

Antiphospholipid syndrome, cancer, pregnancy in third trimester in which fibrinogen levels double, estrogens, chronic myeloproliferative disorders (Polycythemia vera), immobility, ulcerative colitis, and DVT's

Question 40

Saddle embolus

Answer 40

embolus in branch of pulmonary artery (causing pulmonary embolus)

Question 41

What would you expect to find in someone with Monnkenberg's atherosclerosis

Answer 41

Pipestem vessels

Question 42

What congenital defect associated with thrombus in arterial system?

Answer 42

Monckenberg's atherosclerosis

Question 43

Lines of Zahn characteristic of

Answer 43

characteristic in thrombi, particularly in heart or aorta that have alternating bands of RBC's and platelets and fibrin

Question 44

Female patient with family history of DVT's wants to get pregnant. What do you tell her?

Answer 44

Because of family history of clots + pregnancy in third trimester doubles fibrinogen levels, get increased risk of developing clots. Should put her on anticoagulants as prophylaxis treatment to decrease her risk of clots

Question 45

What tests would you order if you suspect someone having some kind of clotting disorder leading to excessive clotting?

Answer 45

Factor V mutation, Antithrombin III level, Protein C and S level, Fibrinogen, Prothrombin (factor II), lupus anticoagulants, anticardiolipin antibodies, Homocysteine, Factor VII- high levels would indicate extrinsic pathway excessive stimulation

Question 46

What can thrombosis in arterial system cause?

Answer 46

Turbulence of blood d/t eddy current, stasis of blood from atrial fibrillation, prevents dilution of coag proteins, and slow flow of blood of coagulation inhibitors

Question 47

What may you find in 5% of people with unexplained miscarriages?

Answer 47

Anticardiolipid antibodies and lupus anticoagulants

Question 48

Predisposing conditions to mural thrombosis

Answer 48

MI, cardiomyopathy, endocarditis, and atrial fibrillation