Bleeding disorders

Question 1

Bleeding patterns in primary and secondary haemostatic failure are the same. true or false?

Answer 1

False

Question 2

Primary haemostatic failure - name 3 causes (broad)

Answer 2

Vascular (vessel wall problem)

Platelets

vWF deficiency

Question 3

Primary haemostatic failure - causes - vascular

Answer 3

Hereditary

Acquired

Question 4

Loss of collagen from the vessel wall increases/decreases with age?

Answer 4

Loss increases with age.

Question 5

Which condition is characterised by a hereditary deficiency of collagen?

Answer 5

Marfan’s syndrome

Question 6

Primary haemostatic failure - causes - platelets

Answer 6

Reduced number

Reduced function

Question 7

Primary haemostatic failure - causes - reduced number of platelets is called

Answer 7

Thrombocytopenia

Question 8

What is the commonest cause of primary haemostatic failure?

Answer 8

Thrombocytopenia

Question 9

Mechanisms of thrombocytopenia

Answer 9

Reduced production

Increased destruction

• DIC
• Autoimmune (ITP)
• hypersplenism

Question 10

Thrombocytopenia - investigations

Answer 10

FBC screening test

- low platelet count

Question 11

Primary haemostatic failure - causes - reduced function of platelets

Answer 11

Drugs (aspirin, NSAIDs)

Renal failure

Question 12

Primary haemostatic failure - causes - vWF deficiency - what mode of inheritance

Answer 12

Autosomal dominant

Question 13

How does vWF deficiency present

Answer 13

Low levels of vWF
Node bleeds
Heavy periods

Question 14

Primary haemostatic failure - clinical features

Answer 14

Spontaneous bruising
- purpura
- petichae 
Mucosal bleeding
- epistaxis 
- GI bleed 
- conjunctival bleed 
- menorrhagia 
Intracranial haemorrhage
Retinal haemorrhage

Question 15

Secondary haemostatic failure - causes

Answer 15

Single clotting factor deficiency

Multiple clotting factor deficiencies

Question 16

Second haemostatic failure - single clotting factor deficiency - name a condition

Answer 16

haemophilia

Question 17

Haemophilia - what is the mode of inheritance

Answer 17

X linked (only affects males)

Question 18

Haemophilia - example of primary or secondary haemostatic failure?

Answer 18

Secondary

Question 19

Haemophilia is a multiple clotting factor deficiency. True or false?

Answer 19

False

- single clotting factor deficiency

Question 20

Haemophilia - definition

Answer 20

Prolonged bleeding recurs episodically at one or more sites on each occasion

Question 21

Haemophilia - patients can’t form ____

Answer 21

Fibrin

Question 22

Haemophilia - there is an abnormality with both primary and secondary haemostasis? true or false

Answer 22

False

- primary haemostasis is normal

Question 23

Haemophilia A - which clotting factor is affected

Answer 23

VIII defieicncy

Question 24

Haemophilia B - which clotting factor is affected

Answer 24

IX defieicny

Question 25

Haemophilia - clinical features

Answer 25

Haemarthrosis (blood in the joint) - inflammation due to iron irritation - neovascularisation Recurrent soft tissue bleeds Prolonged bleeding after dental extractions, surgery

Question 26

Haemophilia - location of bleeding

Answer 26

Usually target joints | - areas of the body that are put under strain (ankle, knee)

Question 27

Haemophilia - PT - APTT

Answer 27

PT = normal APTT = abnormal (prolonged) - since there is low level of VIII or IX

Question 28

Single clotting factor deficiencies are usually hereditary/acquired?

Answer 28

Hereditary

Question 29

Multiple clotting factor deficiencies are usually hereditary/acquired?

Answer 29

Acquired

Question 30

Name 3 causes of multiple clotting factor deficiency

Answer 30

``` Liver failure Vitamin K deficiency Complex coagulopathy (DIC) ```

Question 31

Multiple clotting factor deficiency - Complex coagulopathy

Answer 31

Massive tissue damage leads to excessive activation of haemostatic system. Thrombus forms and gets stuck in the small vessels.

Question 32

Multiple clotting factor deficiency - Complex coagulopathy - there is release of lots of ??

Answer 32

Tissue factor

Question 33

Name 4 conditions which can cause complex coagulopathy

Answer 33

Sepsis RTA Hypovolaemic shock Obstetric emergency

Question 34

Complex coagulopathy - clinical features

Answer 34

Lots of bleeding Bruising Purpura

Question 35

Multiple clotting factor deficiency - Vitamin K defieicny - causes

Answer 35

Poor dietary uptake - low in leafy green veg Malabsorption Warfarin Hemorrhagic disease of newborn

Question 36

Multiple clotting factor deficiency PT APTT

Answer 36

``` PT = prolonged APTT = prolonged ```