Bleeding Disorders

Question 1

Normal haemostatic mechanisms

Answer 1

Vessel wall
Platelets
vWF
Coagulation factors

Question 2

vWF

Answer 2

von Willebrand Factor

Question 3

Primary haemostatic response

Answer 3

Platelet plug formation

Question 4

Secondary haemostatic response

Answer 4

Fibrin plug formation

Question 5

What is haemorrhagic diathesis?

Answer 5

An unusual susceptibility to bleed mostly due to hypocoagulability

Question 6

History of bleeding

Answer 6

Bruising
Epistaxis
Post-surgical bleeding
Menorrhagia
Post-partum haemorrhage
Post-trauma

Question 7

How is severity of bleeding disorders determined?

Answer 7

How appropriate the bleeding is

Question 8

What is the platelet type pattern of bleeding?

Answer 8

Mucosal bleeding
Epistaxis
Purpura
Menorrhagia
GI bleeds

Question 9

What is the coagulation factor pattern of bleeding?

Answer 9

Articular bleeding

Muscle haematoma CNS

Question 10

How can you tell petechiae are petechiae?

Answer 10

They dont blanche

Question 11

What is coagulation factor VIII deficiency?

Answer 11

Haemophilia A

Question 12

What is coagulation factor IX deficiency?

Answer 12

Haemophila B

Question 13

How are haemophilias A and B inherited?

Answer 13

X-linked

Question 14

What is severe haemophila?

Answer 14

Spontaneous bleeding

<1% factor activity

Question 15

What is moderate haemophila?

Answer 15

Bleeds only with provocation (never spontaneous)

1-5% factor activity

Question 16

What is mild haemophilia?

Answer 16

5-30% factor activity

Question 17

Clinical features of haemophilia

Answer 17

Haemarthrosis
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding

Question 18

Which joints are more affected by haemophilia?

Answer 18

Ankle and knee joints - weight bearing
Hip joints less affected
Hinge joints moree affected than ball and socket

Question 19

Complications of haemophilia

Answer 19

Synovitis
Chronic Haemophilic Arthropathy
Neurovascular compression (compartment syndromes)
Other sequelae of bleeding (Stroke)

Question 20

How does haemophilia cause synovitis?

Answer 20

Macrophages in joint eat blood and release cytokines - synovial hypertrophy - lose joint cartilage

Question 21

Haemophilia treatment of bleeding diathesis

Answer 21

Coagulation factor replacement

Prophylaxis in severe haemophilia

Question 22

General haemophilia treatment

Answer 22

Splints
Physiotherapy
Analgesia
Synovectomy
Joint replacement

Question 23

Haemophilia treatment complications

Answer 23

Viral infection (HCV from transfusion before 1987)
DDAVP - MI/hyponatraemia in babies

Question 24

What is the inheritance of von Willebrand disease?

Answer 24

Autosomal dominant

Question 25

What type of bleeding does von Willebrand disease involve?

Answer 25

Platelet type bleeding

Question 26

What is type 1 vW disease?

Answer 26

Quantatitive deficiency

Question 27

What is quantatitive deficiency?

Answer 27

Don't have enough of all of vWF

Question 28

What is type 2 vWD?

Answer 28

Qualitative deficiency - determined by the site of mutation in relation to vWF function

Question 29

What is type 3 vWF function?

Answer 29

Severe (complete) deficiency

Question 30

DDAVP

Answer 30

Desmopressin Used to help stop bleeding in patients with von Willebrand's disease or mild hemophilia A Causes the release of von Willebrand's antigen from the platelets and the cells that line the blood vessels where it is stored

Question 31

What is vWF?

Answer 31

The protein that carries factor VIII

Question 32

Treatment of vW disease

Answer 32

vWF concentrate or DDAVP | OCP etc

Question 33

Acquired bleeding disorders

Answer 33

``` Thrombocytopenia Liver failure Renal failure DIC Drugs ```

Question 34

Causes of thrombocytopenia

Answer 34

Decreased production | Increased consumption

Question 35

What is thrombocytopenia?

Answer 35

Low platelet count

Question 36

Causes of decreased platelet production

Answer 36

Marrow failure Aplasia Infiltration

Question 37

Causes of increased consuption of platelets

Answer 37

Immune ITP Non immune DIC Hypersplenism

Question 38

ITP

Answer 38

Immune thrombocytopenic purpura | A bleeding disorder in which the immune system destroys platelets - blood isolated thrombocytopenia

Question 39

DIC

Answer 39

Disseminated intravascular coagulation Condition in which blood clots form throughout the body, blocking small blood vessels

Question 40

Clinical presentation of thrombocytopenia

Answer 40

Petechia Ecchymosis Mucosal bleeding Rare - CNS bleeding

Question 41

Ecchymosis

Answer 41

Discoloration of the skin resulting from bleeding underneath, typically caused by bruising

Question 42

What is the most likely location of petechiae?

Answer 42

Ankles

Question 43

Associations of ITP

Answer 43

Infection esp EBV, HIV Collagenosis Lymphoma Drug induced

Question 44

Treatment of ITP

Answer 44

Steroids IV IgG Splenectomy Thrombopoietin analogues *NOT transfusion as problem is that immune system is destroying platelets so adding more won’t help*

Question 45

Which clotting factors are made in the liver?

Answer 45

I, II, V, VII, VIII, IX, X, XI

Question 46

APTT

Answer 46

Activated partial thromboplastin time - blood test that characterises coagulation of the blood

Question 47

PT

Answer 47

Prothrombin time | How long it takes the blood to clot

Question 48

PT and APTT in liver failure

Answer 48

Prolonged

Question 49

Vitamin K dependent clotting factors

Answer 49

II, VII, IX, X

Question 50

How is haemorrhagic disease of the newborn prevented?

Answer 50

IM vitamin K administered at birth