6. Disorders of haemostasis

Question 1

what 2 things cause a bleeding disorder?

Answer 1

CF deficiency or dysfunctional CF

Question 2

2 types of bleeding disorder

Answer 2

hereditary or acquired

Question 3

4 types of hereditary bleeding disorder

Answer 3

Haemophilia A, B, C and vW disease

Question 4

2 types of acquired bleeding disorders

Answer 4

consumption or production problem

Question 5

bleeding disorder which is caused by a consumption problem

Answer 5

Disseminated IV coagulation

Question 6

2 production problems in acquired bleeding disorder

Answer 6

Vit K def, Liver disease

Question 7

8 main sxs of bleeding disorders

Answer 7

bleeding gums
purpura
chronic anaemia 
mennorhagia 
bruising 
petichae rash
joint bleeds 
muscle bleeds

Question 8

what is Haemophilia A?

Answer 8

FVIII deficiency

Question 9

what is Haemophilia B?

Answer 9

FIX deficiency

Question 10

what is Haemophilia C?

Answer 10

FXI deficiency

Question 11

5 CF examples which are autosomal recessive

Answer 11

prothrombin = IIa
fibrinogen = I 
FV
FVII
FXIII

Question 12

where are autosomal reccessive CF disorders common?

Answer 12

countries with incest

Question 13

3 causes of vitamin K deficiency

Answer 13

1. inadequate diet
2. malabsorption
3. drugs (warfarin)

Question 14

how does vitamin K deficiency affect haemostasis?

Answer 14

leads to decreased synthesis of F II, VII, IX, X

Question 15

what is the APTT and PT in vit K def? why?

Answer 15

prolonged in both - as many factors involved which are part of instrinsic and extrinsic

Question 16

what does liver disease cause?

Answer 16

impaired absorption of Vit K

Question 17

how does liver diease affect haemostasis?

Answer 17

same effects of Vit K deficiency - leads to decreased synthesis of F II, VII, IX, X

Question 18

what is desseminated intravascular coagulation?

Answer 18

blood clots block small vessels

Question 19

how does DIC affect haemostasis?

Answer 19

excessive activation of tissue factor - therefore excessive coagulation

Question 20

why can DIC lead to amputation?

Answer 20

deposition of fibrin in microcirculation

Question 21

what is acquired haemophilia

Answer 21

inhibits factor VIII

Question 22

who is acquired haemophilia prevalent in?

Answer 22

elderly pt with underlying malignant disease

Question 23

2 types of platelet disorders

Answer 23

hereditary or acquired

Question 24

3 hereditary platelet disorders

Answer 24

1. glanzmann’s thromboasthenia
2. Bernard Soulier syndrome
3. vW disease

Question 25

3 acquired platelet disorder types

Answer 25

1. dysfuntional 2. shortened lifespan 3. production problems

Question 26

what can cause dysfunctional acquired platelet disorders?

Answer 26

drugs

Question 27

what can cause shortened lifespan platelet disorders?

Answer 27

malaria, bleeding

Question 28

what can cause platelet production disorders?

Answer 28

leukaemia post-viral infection immunosuppression

Question 29

examples of drugs which affect platelet function (10) 4 are not drugs

Answer 29

1. caffeine 2. NSAIDS/aspirin 3. CV 4. antimicrobials 5. anti-coagulants 6. chemotherapy drugs 7. onions/garlic 8. Vit E 9. alcohol 10. fish oil

Question 30

2 types of thrombotic disorders

Answer 30

thrombosis and hereditary thrombophilia

Question 31

2 types of thrombosis

Answer 31

arterial and venous

Question 32

4 examples of hereditary thrombophilia

Answer 32

1. FV leiden mutation 2. anti-thrombin deficiency 3. protein S and C deficiency 4. prothrombin gene mutation

Question 33

what is thrombosis?

Answer 33

formation of a solid mass (thrombus), in the lumen of a blood vessel

Question 34

what is a thrombus formed from

Answer 34

platelets and fibrin

Question 35

what 4 diseases are associated with thrombi?

Answer 35

1. MI 2. cerebral vascular disease 3. Deep vein thrombosis 4. pulmonary embolism

Question 36

describe pathogenesis of arterial thrombosis

Answer 36

1. atherosclerosis 2. injury to vessel 3. blood leaks out and comes into contact with subendothelial collagen and TF 4. primary haemostasis - formation of platelet plug 5. thrombus blocks artery

Question 37

6 risk factors for arterial thrombosis

Answer 37

1. diabetes 2. smoking 3. raised BP 4. raised lipids 5. male 6. family history

Question 38

pathogenesis of venous thrombosis

Answer 38

1. increased coagubility of blood 2. blood flow slows 3. coagulation at site of initiation of thrombus

Question 39

4 main risk factoes for venous thrombosis

Answer 39

1. FV leiden gene mutation 2. Blood group 3. prothombin gene mutation 4. protein S and C deficiency

Question 40

which type of thrombosis is caused by immobility?

Answer 40

venous

Question 41

what is the FV leiden mutation?

Answer 41

Arg replaced with glut - pos 560 on FV gene

Question 42

is antithrombin def autosomal dominant or recessive?

Answer 42

dominant

Question 43

how to treat antithrombin def?

Answer 43

anti-thrombin deficiency

Question 44

what are the effcts of prothrombin gene mutation?

Answer 44

increased levels of circualting prothrombin

Question 45

what is the prothrombin gene mutation?

Answer 45

Guan replaced by adenine - pos 20210

Question 46

role of protein S

Answer 46

co-factor to protein C

Question 47

is protien S def autosomal dominant or recessive?

Answer 47

dominant

Question 48

normal role of protein C

Answer 48

activated by thrombin | cleaves FVa and FVIII to inactivate them - prevents excessive clotting

Question 49

5 ways of treating thrombosis

Answer 49

1. heparin 2. warfarin 3. FXa inhibitors 4. direct thombin inhibitors 5. anti-platelet drugs

Question 50

how is heparin given

Answer 50

IV

Question 51

how is warfarin given

Answer 51

orally

Question 52

give an example of FXa inihibitor

Answer 52

rivaroxaban

Question 53

which direct thrombin inhibitor replaces heparin?

Answer 53

Bivalirudin

Question 54

which direct thrombin inhibitor replaces warfarin?

Answer 54

dabigatran

Question 55

give an example of an antiplatelet drug

Answer 55

aspirin