Module 4: Disorders of Hemostasis (from review- Coagulation disorders)

Question 1

coagulation disorders are due to (4)

Answer 1

Failure of synthesis
Production of abnormal molecules
Excessive destruction or consumption of factors
Inactivation of factor by circulating anticoagulants

Question 2

Screening tests

Answer 2

PT

PTT

Question 3

PT test is for which factors

Answer 3

Extrinsic
Factors II, V, VII, X, I

Silly
Sluts
Love
Pillow
Fights

Question 4

PTT test is for which factors

Answer 4

Intrinsic
All factors except III, VII, XIII

Horney
Prostitutes
Have
Coitus
And
Spread
Legs
For
Precious
Pennies

Question 5

Inherited disorders

Answer 5

all factors can be deficient

Question 6

3 common inherited disorders

Answer 6

Hemophilia A (factor VIIIc)
Hemophilia B (factor IX), christmas disease
Von Willebrands Disease

Question 7

Severity of bleeds %

Answer 7

For factors IX, VIII, X, V, II, I

Severe

Question 8

What factors cause a marked prolonged PTT but no clinical bleeding (3)

Answer 8

XII, HMWK, PK

Question 9

What factors cause a disproportionate mild bleed (2)

Answer 9

XI, VII

Question 10

What factors cause a disproportionate severe bleed (1)

Answer 10

XIII

Question 11

Normal male and normal female

Answer 11

children all normal unless there is a mutation

Question 12

Normal male and carrier female (undiagnosed)

Answer 12

most common combination which perpetuates the disease

produces XhX, XX, XhY, XY

50% normal (male and female)
25% carriers (female)
25% hemophiliacs (male)

Question 13

What is the most common bleeding disorder

Answer 13

von willebrands disease

Question 14

what is the most common hemophilia

Answer 14

Hemophilia A

Question 15

Hemophilia A

Answer 15

Factor deficient: VIIIc
Gene affected: Xq28
Therapy: factor VIII infusion
Complication: development of immune VIII antibodies

Question 16

Hemophilia B

Answer 16

Factor deficient: IX
Gene affected:Xq27
Therapy: Prothrombin group concentrate or IX concentrate

Question 17

Which hemophilia therapy lasts longer and why

Answer 17

IX because of its longer half-life

Therapy is given less often which reduced the chance of developing antibodies against the therapy

Question 18

What type of disorder is Von Willebrands disease

Answer 18

platelet adhesion

Question 19

von Willebrands Disease

Answer 19

What chromosome is affected: 12
Treatment: VIII concentrate
Tests for workup: Plt agglutination with ristocetin, plt aggregation with ristocetin (reduced)

Question 20

3 types of von Willebrands Disease

Answer 20

Type 1 (most common)
Type 2
Type 3

Question 21

Lab findings for Hemophilia A

Answer 21

Plt count: normal
Bleeding time: normal
PT: normal
PTT: prolonged

Question 22

Lab findings for Hemophilia B

Answer 22

Plt count: normal
Bleeding time: normal
PT: normal
PTT: prolonged

Question 23

Lab findings for von Willebrands Disease, Type 1

Answer 23

Plt count: normal
Bleeding time: usually prolonged
PT: normal
PTT: usually normal but may be prolonged

Question 24

Acquired coagulation deficiencies (5 types)

Answer 24

Much more common than inherited
Circulating abnormal anticoagulants and antifactors
Vitamin K deficiency
Liver Disease
DIC
Disorders of Fibrinolysis

Question 25

Circulating abnormal anticoagulants and antifactors

Answer 25

A) specific factor antibodies B) non-specific inhibitors "antiphospholipid antibody" -Lupus anticoagulant -Anticardiolipin antibody Test used to differentiate deficiency from circulating anticoagulants or antifactors: mixing study

Question 26

Vitamin K deficiency

Answer 26

factors are not caboxylated and cannot bind calcium Factors involved: Prothrombin group (II, VII, IX, X) and Protein C and S PIVKA stands for: Proteins induced by vitamin K absence = non carboxylated, inactive abnormal prothrombin factor molecules Oral anticoagulant: Warfarin (Coumadin)

Question 27

Liver Disease

Answer 27

reduced production in diseased hepatic cells Factors involved: II, VII, IX, X, V, I

Question 28

2 liver disorders that affect coagulation

Answer 28

Obstructive jaundice :cannot absorb Vitamin K Severe hepatitis: Damaged cells produce less factor

Question 29

3 things destruction of liver cells may result in

Answer 29

DIC: coagulation factors are not removed Released increased amounts of tPA from hepatic cell triggering fibrinolysis Spenomegaly, increasing splenic sequestering of platelets

Question 30

DIC results in

Answer 30

widespread intravascular coagulation and secondary fibrinolysis Results in: consumption and destruction of coag factors until they are deficient; thrombocytopenia; abnormal bleeding

Question 31

causes of DIC

Answer 31

any situation which causes exposure of a lot of foreign surfaces to the blood, or the widespread release of coagulation activators (FIII) or activators of fibrinolysis

Question 32

DIC laboratory findings should confirm (4)

Answer 32

activation of coagulation activation of fibrinolysis inhibitor consumption end organ damage or failure

Question 33

Main lab results for DIC

Answer 33

``` Factor deficiencies (cause prolonged PT/PTT) Thrombocytopenia Increased fdp, FDP Increased D-dimer Schistocytes (broken up RBC) ```

Question 34

Disorders of fibrinolysis

Answer 34

Primary: activation of fibrinolysis in the absence of coagulation Secondary: whenever fibrin is deposited as normal (but if abnormal DIC) Therapeutic: injection of direct plasminogen activators Impaired Fibrinolysis: due to decreased production, increased destruction or increased inhibition of plasminogen, plasminogen activators or plasmin

Question 35

Differentiate primary from secondary fibrinolysis

Answer 35

Fibrinogen degradation product test: Primary = pos Secondary = pos D dimer test: Fibrin degradation products: Primary = NEG secondary = pos

Question 36

Thrombosis is

Answer 36

abnormal formation in circulation of solid, localized masses of fibrin and/or platelets that cause partial or complete blockage of vessels

Question 37

Thromboembolism

Answer 37

thrombi break away and block smaller vessels

Question 38

pulmonary embolism is

Answer 38

obstruction of pulmonary artery or branch of | Usually from DVT (deep vein thrombosis)

Question 39

3 factors involved in forming a thrombus are

Answer 39

slowed blood flow hypercoagulability vessel wall damage

Question 40

thrombi

Answer 40

multiple blood clots

Question 41

thrombus

Answer 41

single blood clot

Question 42

Inherited disorders

Answer 42

``` Factor V leiden gene mutation (protein C resistance) Antithrombin III Protein C or S deficiency Homocysteinuria Dysfibrinogenemia Abnormal plasminogen Prothrombin G20210A Variant ```

Question 43

What immune condition is an acquired coagulation deficiency and also an acquired disorder that promotes thrombosis

Answer 43

lupus anticoagulant

Question 44

Factor V Leiden gene mutation (protein C resistance)

Answer 44

most common cause of increased risk of venous thrombosis Produce an abnormal factor V molecule that resists the action of activated protein C, thus the factor is inappropriately maintained in an active form and therefore continues to promote coagulation

Question 45

Antithrombin III

Answer 45

normally works with heparin to inhibit factors Inadequate inhibition of thrombin, Xa and IXa

Question 46

Protein C or S deficiency

Answer 46

inadequate inhibition of V and VIII | impaired activation of fibrinolysis because activated protein C inhibits antiplasmin

Question 47

Homocysteinuria

Answer 47

damaged endothelium by deposits of amino acid causes activation of coagulation and atherosclerosis

Question 48

Dysfibrinogenemia

Answer 48

abnormal fibrinogen makes abnormal fibrin that will not neutralize thrombin

Question 49

Abnormal plasminogen

Answer 49

impaired formation of plasmin

Question 50

Prothrombin G20210A variant

Answer 50

abnormal allele of II = increased II levels = increased thrombosis