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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Normal haemostatic mechanisms

A

Vessel wall
Platelets
vWF
Coagulation factors

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2
Q

vWF

A

von Willebrand Factor

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3
Q

Primary haemostatic response

A

Platelet plug formation

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4
Q

Secondary haemostatic response

A

Fibrin plug formation

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5
Q

What is haemorrhagic diathesis?

A

An unusual susceptibility to bleed mostly due to hypocoagulability

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6
Q

History of bleeding

A 
Bruising
Epistaxis
Post-surgical bleeding
Menorrhagia
Post-partum haemorrhage
Post-trauma
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7
Q

How is severity of bleeding disorders determined?

A

How appropriate the bleeding is

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8
Q

What is the platelet type pattern of bleeding?

A 
Mucosal bleeding
Epistaxis
Purpura
Menorrhagia
GI bleeds
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9
Q

What is the coagulation factor pattern of bleeding?

A

Articular bleeding

Muscle haematoma CNS

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10
Q

How can you tell petechiae are petechiae?

A

They dont blanche

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11
Q

What is coagulation factor VIII deficiency?

A

Haemophilia A

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12
Q

What is coagulation factor IX deficiency?

A

Haemophila B

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13
Q

How are haemophilias A and B inherited?

A

X-linked

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14
Q

What is severe haemophila?

A

Spontaneous bleeding

<1% factor activity

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15
Q

What is moderate haemophila?

A

Bleeds only with provocation (never spontaneous)

1-5% factor activity

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16
Q

What is mild haemophilia?

A

5-30% factor activity

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17
Q

Clinical features of haemophilia

A 
Haemarthrosis
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding
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18
Q

Which joints are more affected by haemophilia?

A

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

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19
Q

Complications of haemophilia

A

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

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20
Q

How does haemophilia cause synovitis?

A

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

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21
Q

Haemophilia treatment of bleeding diathesis

A

Coagulation factor replacement

Prophylaxis in severe haemophilia

22
Q

General haemophilia treatment

A 
Splints
Physiotherapy
Analgesia
Synovectomy
Joint replacement
23
Q

Haemophilia treatment complications

A 
Viral infection (HCV from transfusion before 1987)
DDAVP - MI/hyponatraemia in babies
24
Q

What is the inheritance of von Willebrand disease?

A

Autosomal dominant

25
Q

What type of bleeding does von Willebrand disease involve?

A

Platelet type bleeding

26
Q

What is type 1 vW disease?

A

Quantatitive deficiency

27
Q

What is quantatitive deficiency?

A

Don’t have enough of all of vWF

28
Q

What is type 2 vWD?

A

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

29
Q

What is type 3 vWF function?

A

Severe (complete) deficiency

30
Q

DDAVP

A

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

31
Q

What is vWF?

A

The protein that carries factor VIII

32
Q

Treatment of vW disease

A

vWF concentrate or DDAVP

OCP etc

33
Q

Acquired bleeding disorders

A 
Thrombocytopenia
Liver failure
Renal failure
DIC
Drugs
34
Q

Causes of thrombocytopenia

A

Decreased production

Increased consumption

35
Q

What is thrombocytopenia?

A

Low platelet count

36
Q

Causes of decreased platelet production

A

Marrow failure
Aplasia
Infiltration

37
Q

Causes of increased consuption of platelets

A

Immune ITP
Non immune DIC
Hypersplenism

38
Q

ITP

A

Immune thrombocytopenic purpura

A bleeding disorder in which the immune system destroys platelets - blood isolated thrombocytopenia

39
Q

DIC

A

Disseminated intravascular coagulation

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

40
Q

Clinical presentation of thrombocytopenia

A

Petechia
Ecchymosis
Mucosal bleeding
Rare - CNS bleeding

41
Q

Ecchymosis

A

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

42
Q

What is the most likely location of petechiae?

A

Ankles

43
Q

Associations of ITP

A

Infection esp EBV, HIV
Collagenosis
Lymphoma
Drug induced

44
Q

Treatment of ITP

A

Steroids
IV IgG
Splenectomy
Thrombopoietin analogues

NOT transfusion as problem is that immune system is destroying platelets so adding more won’t help

45
Q

Which clotting factors are made in the liver?

A

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

46
Q

APTT

A

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

47
Q

PT

A

Prothrombin time

How long it takes the blood to clot

48
Q

PT and APTT in liver failure

A

Prolonged

49
Q

Vitamin K dependent clotting factors

A

II, VII, IX, X

50
Q

How is haemorrhagic disease of the newborn prevented?

A

IM vitamin K administered at birth

Haematology (18 decks)

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