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HEMATOLOGY > Coagulation Disorders > Flashcards

Coagulation Disorders Flashcards

1
Q

Thromboplastin aka

A

Tissue Factor

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2
Q

Mechanism of Warfarin

A

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

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3
Q

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

A

Intrinsic pathway- 28-30 seconds

Extrinsic pathway- 9-12 seconds

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4
Q

Factor IX aka

A

Christmas factor

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5
Q

What are the hereditary bleeding coagulation disorders

A

hemophilias, afibrinogenemia, clotting factor deficiencies

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6
Q

How is hemophilia A inherited?

A

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

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7
Q

How is hemophilia B inherited?

A

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

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8
Q

Manifestation if 5-75% of normal Factor VIII level

A

mild bleeding

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9
Q

Manifestation if 1-5% of normal Factor VIII level

A

moderate bleeding

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10
Q

Manifestation if less than 1% of normal Factor VIII level

A

Severe bleeding

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11
Q

Hemophilia A and B clinical findings

A

Easy bruising, spontaneous bleeding, hemorrhages, and hemarthrosis

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12
Q

1 unit of Factor VIII

A

equal to 100ng in 1 ml of plasma

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13
Q

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

A

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

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14
Q

Complication of giving Factor VIII concentrates

A

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

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15
Q

Tx options for hemophilia A

A

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

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16
Q

DDAVP mechanism

A

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

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17
Q

EACA

A

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

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18
Q

Benefits of prophylaxis of Factor VIII concentrates in hemophilia A

A

Reduces detectable joint abnormalities

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19
Q

Which factor deficiency is not really associated with bleeding problems?

A

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

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20
Q

Hemophilia most common in Ashkenazi jews

A

C

21
Q

Tx for afibrinogenemia

A

cryoprecipitate

22
Q

which hemophilia causes mild bleeding post-op?

A

Hemophilia C

23
Q

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

A

DIC

24
Q

Diagnosis of DIC

A

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

25
Q

Causes of DIC

A

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

26
Q

Tx of DIC

A

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

27
Q

Acquired causes of coagulation bleeding disorders

A

Vitamin K deficiency, Liver disease, DIC

28
Q

What effects would liver disease have on clotting?

A

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

29
Q

Tx for liver disease to replace clotting factors

A

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

30
Q

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?

A

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)

31
Q

What is the major culprit in causing DIC

A

Uncontrolled thrombin generation leading to small small clots in microcirculation

32
Q

Inherited coag disorders resulting in too much clotting

A

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

33
Q

How is antithrombin III deficiency inherited?

A

Autosomal dominant

34
Q

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

A

Factor V Leiden

35
Q

How is factor V leiden inherited?

A

Can be homozygous or heterozygous- mutation in factor V gene

36
Q

How is protein C deficiency inherited

A

autosomal dominant

37
Q

2 types of protein C deficiency

A

Type I- quantitiative

Type II- qualitative

38
Q

antiphospholipid syndromes characterized by

A

anticardiolipin antibodies or lupus anticoagulants

39
Q

Non-inherited causes of hypercoagulable states leading to increased clotting

A

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

40
Q

Saddle embolus

A

embolus in branch of pulmonary artery (causing pulmonary embolus)

41
Q

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

A

Pipestem vessels

42
Q

What congenital defect associated with thrombus in arterial system?

A

Monckenberg’s atherosclerosis

43
Q

Lines of Zahn characteristic of

A

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

44
Q

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

A

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

45
Q

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

A

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

46
Q

What can thrombosis in arterial system cause?

A

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

47
Q

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

A

Anticardiolipid antibodies and lupus anticoagulants

48
Q

Predisposing conditions to mural thrombosis

A

MI, cardiomyopathy, endocarditis, and atrial fibrillation

HEMATOLOGY (5 decks)

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