Goljan Pathology Chapter 14

Front 1

Heparin-like molecules. Function?

Back 1

Enhance antithrombin III (ATIII) activity.

Front 2

Antithrombin III. Function?

Back 2

Neutralizes activated serine protease coagulation factors.

Front 3

PGI2 (prostacyclin). Function (2)?

Back 3

1) Vasodilator
2) Inhibits platelet aggregation

Front 4

Proteins C and S. Function (2)?

Back 4

1) Inactivate factors V and VIII
2) Enhance fibrinolysis

Front 5

tPA. Function?

Back 5

Activates plasminogen to release plasmin

Front 6

Thromboxane A2 (TXA2). Function (2)?

Back 6

1) Vasoconstrictor
2) Enhances platelet aggregation

Front 7

vWF. Two functions? Synthesized where?

Back 7

a) platelet adhesion, b) prevents degradation of VIII:c in plasma
Synthesized in Weibel-Palade bodies in endothelial cells

Front 8

Factor VIII:c. Synthesized where (2)?

Back 8

1) Liver
2) Reticuloendothelial cells

Front 9

In the extrinsic coagulation pathway, what activates factor VII?

Back 9

Tissue factor (aka tissue thromboplastin)

Front 10

List the important platelet receptors and their cognate ligands (2).

Back 10

1) GpIb, vWF
2) GpIIb-IIIa, fibrinogen

Front 11

What is the function of clopidogrel?

Back 11

Inhibits expression of GpIIb-IIIa, decreasing platelet aggregation.

Front 12

What are three important compounds are stored in platelets?

Back 12

1) ADP
2) vWF
3) fibrinogen

Front 13

What molecule is involved in the first step of the extrinsic pathway?

Back 13

Factor VII

Front 14

What factor is involved in the first step of the intrinsic pathway?

Back 14

Factor XII (Hageman factor)

Front 15

Which factors are vitamin K dependent (4)?

Back 15

1972
factors 10, 9, 7, and 2

Front 16

What three compounds can activate plasminogen?

Back 16

1) tPA
2) streptokinase (from streptococci)
3) urokinase (from human urine)

Front 17

What are D-dimers?

Back 17

Cross-linked fibrin monomers

Front 18

What is the sequence of events that a platelet goes through in hemostasis of small vessel injury (4)?

Back 18

1) adhesion
2) granule release
3) synthesis of TXA2
4) temporary plug formation

Front 19

What holds the temporary platelet plug together (1)?

Back 19

fibrinogen

Front 20

A temporary plug is converted to a stable plug by the action of ______, which converts _______ to ______.

Back 20

a) thrombin
b) fibrinogen
c) fibrin

Front 21

What does PTT test?

Back 21

The intrinsic system.

Front 22

What does PT test?

Back 22

The extrinsic system.

Front 23

What does D-dimer assay test?

Back 23

Fibrin split products. High sensitivity, low specificity.

Front 24

What two findings do TTP and HUS have in common?

Back 24

1) platelet consumption
2) hemolytic anemia with schistocytes

Front 25

What are some common causes of thrombocytosis (3)?

Back 25

1) chronic iron deficiency
2) malignancy
3) splenectomy

Front 26

What is the most common cause of a qualitative (functional) platelet defect?

Back 26

Aspirin

Front 27

What physical exam sign is only seen with thrombocytopenia?

Back 27

Petechiae

Front 28

Clinical findings in coagulation disorders (2).

Back 28

1) late rebleeding (no stable platelet plug formation)
2) hemarthroses (severe deficiency)

Front 29

What is the mode of inheritance of hemophilia A?

Back 29

X-linked recessive

Front 30

What is the defect in hemophilia A?

Back 30

Factor VIII (intrinsic pathway)

Front 31

What is the mode of inheritance of hemophilia B?

Back 31

X-linked recessive

Front 32

What is the defect in hemophilia B?

Back 32

Factor IX deficiency (intrinsic pathway)

Front 33

What are laboratory findings for hemophilia A (2)?

Back 33

1) decreased VIII:c
2) increased PTT

Front 34

What is the most common hereditary coagulation disorder?

Back 34

von Willebrand disease

Front 35

What is the mode of transmission of von Willebrand disease?

Back 35

Autosomal dominant

Front 36

What are the functional abnormalities in vWD (2)?

Back 36

1) platelet aggregation disorder (↓ vWF antigen)
2) coagulation factor disorder (↓ VIII:c activity)

Front 37

What laboratory tests will be abnormal in vWD (2)?

Back 37

1) ↑ PTT
2) ↑ bleeding time

Front 38

What two important circumstances may lead to vitamin K deficiency?

Back 38

1) Newborns with no bacteria
2) Hospitalized patients treated with antibiotics

Front 39

What does warfarin inhibit?

Back 39

epoxide reductase

Front 40

Liver cirrhosis leads to _____ synthesis of vitamin K-dependent factors and _____ activation of vitamin K.

Back 40

a) decreased
b) decreased

Front 41

(True/False) Liver cirrhosis leads to many hemostasis abnormalities leading to increased PT, PTT, bleeding time, and D-dimers.

Back 41

True

Front 42

What is the most common cause of DIC?

Back 42

Sepsis

Front 43

What type of coagulopathy is DIC?

Back 43

Consumptive

Front 44

Why does DIC cause thrombosis and hemorrhage?

Back 44

Because it activates the coagulation cascade (thrombosis), but due to consumption of platelets, leads to hemorrhage.

Front 45

What are two common anti-phospholipid antibodies?

Back 45

1) anticardiolipin antibody
2) lupus anticoagulant

Front 46

What antibody gives a false positive syphilis test?

Back 46

anticardiolipin antibody

Front 47

What is the most common hereditary thrombosis syndrome?

Back 47

Factor V leiden