Bleeding Disorders

Question 1

Vascular abnormality causes of failure of platelet plug (4)

Answer 1

Hereditary e.g. Marfan’s,

acquired e.g. vasculitis - HSP, ageing, vitamin C deficiency

Question 2

Primary haemostasis bleeding pattern

Answer 2

purpura, 
petechiae
mucosal blood blisters, 
retinal haemorrhages, 
mucosal type bleeding, 
epistaxis, 
easy bleeding,
menorrhagia

Question 3

Thrombocytopaenia causes

Answer 3

hereditary - rare,

acquired - reduced production e.g. marrow problems, increased peripheral destruction

Question 4

How does marrow problem causing platelet forming problems usually present on bloods?

Answer 4

pancytopenia with reduced Hb and reduced white cells

Question 5

Majority of causes of thrombocytopaenia are hereditary. T/F?

Answer 5

False - mostly acquired and mostly increased destruction

Question 6

Causes of peripheral platelet destruction (3)

Answer 6

Coagulopathy e.g. DIC, when coagulating pathway activated,
autoimmune e.g. immune thrombocytopenia purpura (ITP),
hypersplenism e.g. liver disease

Question 7

Commonest cause of thrombocytopaenia

Answer 7

ITP - peripheral platelets destructed by immune process

Question 8

Platelet function defects causes

Answer 8

hereditary (rare),

acquired e.g. drugs - aspirin, NSAIDs, renal failure,

Question 9

vWF deficiency causes

Answer 9

acquired (rare),

hereditary - generally mild

Question 10

hereditary vWF is usually autosomal dominant. T/F?

Answer 10

True

Question 11

Commonest cause of primary haemostatic failure, is primary haemostatic failure usually hereditary or acquired?

Answer 11

thrombocytopaenia,

usually acquired

Question 12

Failure of fibrin clot formation causes

Answer 12

multiple clotting factor deficiencies,

single clotting factor deficiency

Question 13

Multiple clotting factor deficiency is usually acquired. Give 3 examples of multiple clotting factor deficiency causes?

Answer 13

liver failure,
vitamin K deficiency/warfarin therapy,
complex coagulopathy e.g. DIC

Question 14

Single clotting factor deficiency is usually hereditary. What is the most common

Answer 14

Haemophilia A and B i.e. clotting factors 8 and 9

Question 15

PT and APTT results in multiple factor deficiencies?

Answer 15

both delayed

Question 16

complex coagulopathy meaning

Answer 16

everything activated and used up in coagulation pathway

Question 17

Where are all coagulation factors synthesised?

Answer 17

Hepatocytes

Question 18

What factors need vitamin K for carboxylation?

Answer 18

II, VII, IX and X

Question 19

Sources of vitamin K (2)

Answer 19

diet (leafy green veg),

intestinal synthesis

Question 20

Where is vitamin K absorbed and what does it need for absorption

Answer 20

absorbed in upper intestine,

needs bile salts for absorption

Question 21

Causes of vitamin K deficiency (5)

Answer 21

poor dietary intake, 
malabsorption e.g. Crohn's, 
obstructive jaundice, 
warfarin, 
haemorrhagic disease of the newborn

Question 22

Mechanism of action of warfarin

Answer 22

Vitamin K antagonists

Question 23

What causes haemorrhagic disease of the newborn? How prevented?

Answer 23

dietary intake doesn’t contain it,
bowels aren’t yet making it,
preventing by IM vit K at birth

Question 24

What is disseminated intravascular coagulation

Answer 24

excess and inappropriate activation of the primary, secondary and fibrinolytic haemostatic system

Question 25

What forms in DIC leading to end organ failure?

Answer 25

microvascular thrombus formation

Question 26

DIC can occur where there is excessive tissue damage leading to haemostasis activation. Give 4 examples of causes of DIC

Answer 26

sepsis, cancer, trauma e.g. RTA so hypovolaemic shock, obstetric emergencies e.g. placental abruption,

Question 27

How does body try to compensate in DIC and what does this cause in the patient?

Answer 27

fibrinolysis so clotting factor consumption to try and break up all the microvascular thrombi but then platelets are consumed, this causes bruising, purpura and generalised bleeding

Question 28

What will be raised in bloods in DIC?

Answer 28

FDPs i.e. D-Dimers

Question 29

Treatment of DIC

Answer 29

treat underlying cause, | replacement therapy including platelets, plasma and fibrinogen

Question 30

Haemophilia pattern of inheritance? What does this mean?

Answer 30

X-linked so females don't have it severely

Question 31

Haemophilia presentation (4)

Answer 31

recurrent haemarthroses (ankles most commonly, knees, elbows, often same joint), recurrent soft tissue bleeds e.g. bruising in toddlers, FH testing, prolonged bleeding after dental extractions/surgery

Question 32

Haemophilia A

Answer 32

factor VIII deficiency

Question 33

Haemophilia B

Answer 33

factor IX deficiency

Question 34

Haemophilia B is 5 times more common than Haemophilia A. T/F?

Answer 34

False - A 5 times more common than B

Question 35

Haemophilia patients don't get to get prolonged bleeding from small cuts like paper cuts. T/F?

Answer 35

true

Question 36

Types of haemophilia and which most common

Answer 36

mild, moderate, severe - severe most common

Question 37

PT and APTT screening tests results in haemophilia

Answer 37

PT normal, | APTT abnormal

Question 38

Haemophilia and recurrent haemarthroses can damage joints over time. T/F?

Answer 38

True

Question 39

Treatment for haemophilia

Answer 39

Intravenous clotting factors

Question 40

Echiymosis - what is it and what does it indicate?

Answer 40

big bruises that are bigger than they should be for injury, indicates problem with coagulation cascade

Question 41

DIC screen

Answer 41

``` FBC, APTT, PT, fibrinogen, (especially), D-dimer (especially) ```

Question 42

DIC presentation

Answer 42

``` large bruising, bleeding from venepuncture sites, confusion, fever, petechiae & purpura, ARDs ```

Question 43

Why do alcoholics end up with thrombocytopaenia?

Answer 43

toxic to bone marrow and also could have hypersplenism and big liver where platelets can get stuck