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

HEMA 256 MB > Module 4: Disorders of Hemostasis (from review- Coagulation disorders) > Flashcards

Flashcards in Module 4: Disorders of Hemostasis (from review- Coagulation disorders) Deck (50):
1

coagulation disorders are due to (4)

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

2

Screening tests

PT
PTT

3

PT test is for which factors

Extrinsic
Factors II, V, VII, X, I

Silly
Sluts
Love
Pillow
Fights

4

PTT test is for which factors

Intrinsic
All factors except III, VII, XIII

Horney
Prostitutes
Have
Coitus
And
Spread
Legs
For
Precious
Pennies

5

Inherited disorders

all factors can be deficient

6

3 common inherited disorders

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

7

Severity of bleeds %

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

Severe

8

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

XII, HMWK, PK

9

What factors cause a disproportionate mild bleed (2)

XI, VII

10

What factors cause a disproportionate severe bleed (1)

XIII

11

Normal male and normal female

children all normal unless there is a mutation

12

Normal male and carrier female (undiagnosed)

most common combination which perpetuates the disease

produces XhX, XX, XhY, XY

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

13

What is the most common bleeding disorder

von willebrands disease

14

what is the most common hemophilia

Hemophilia A

15

Hemophilia A

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

16

Hemophilia B

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

17

Which hemophilia therapy lasts longer and why

IX because of its longer half-life

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

18

What type of disorder is Von Willebrands disease

platelet adhesion

19

von Willebrands Disease

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

20

3 types of von Willebrands Disease

Type 1 (most common)
Type 2
Type 3

21

Lab findings for Hemophilia A

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

22

Lab findings for Hemophilia B

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

23

Lab findings for von Willebrands Disease, Type 1

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

24

Acquired coagulation deficiencies (5 types)

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

25

Circulating abnormal anticoagulants and antifactors

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

26

Vitamin K deficiency

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)

27

Liver Disease

reduced production in diseased hepatic cells

Factors involved: II, VII, IX, X, V, I

28

2 liver disorders that affect coagulation

Obstructive jaundice :cannot absorb Vitamin K

Severe hepatitis: Damaged cells produce less factor

29

3 things destruction of liver cells may result in

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

30

DIC results in

widespread intravascular coagulation and secondary fibrinolysis

Results in: consumption and destruction of coag factors until they are deficient; thrombocytopenia; abnormal bleeding

31

causes of DIC

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

32

DIC laboratory findings should confirm (4)

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

33

Main lab results for DIC

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

34

Disorders of fibrinolysis

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

35

Differentiate primary from secondary fibrinolysis

Fibrinogen degradation product test:
Primary = pos
Secondary = pos

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

36

Thrombosis is

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

37

Thromboembolism

thrombi break away and block smaller vessels

38

pulmonary embolism is

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

39

3 factors involved in forming a thrombus are

slowed blood flow
hypercoagulability
vessel wall damage

40

thrombi

multiple blood clots

41

thrombus

single blood clot

42

Inherited disorders

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

43

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

lupus anticoagulant

44

Factor V Leiden gene mutation (protein C resistance)

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

45

Antithrombin III

normally works with heparin to inhibit factors

Inadequate inhibition of thrombin, Xa and IXa

46

Protein C or S deficiency

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

47

Homocysteinuria

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

48

Dysfibrinogenemia

abnormal fibrinogen makes abnormal fibrin that will not neutralize thrombin

49

Abnormal plasminogen

impaired formation of plasmin

50

Prothrombin G20210A variant

abnormal allele of II = increased II levels = increased thrombosis