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Haematology Week 2 2018/19 > 3: Bleeding disorders > Flashcards

3: Bleeding disorders Flashcards

1
Q

What are some causes of problems with secondary haemostasis?

A

Single factor deficiencies - Haemophilia A & B

Multiple factor deficiencies - Vitamin K deficiency, liver disease, DIC

note that DIC is a cause of problems with both primary and secondary haemostasis (because it drains platelets AND clotting factors)

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2
Q

What are some causes of problems with primary haemostasis?

A

VASCULAR: less available collagen (ageing), Vit C deficiency, steroids, Henoch Schonlein purpura (vasculitis)

PLATELETS: bone marrow suppression, renal failure, autoimmune thrombocytic purpura, DIC, hypersplenism

VON WILLEBRAND DISEASE

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3
Q

What is a

reduced

increased

number of platelets called?

A

Reduced platelets - thrombocytopaenia

Increased platelets - thrombocytosis

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4
Q

Which drugs reduce the function of platelets?

A

Anti-platelets e.g aspirin, clopidogrel, ticagrelor

NSAIDs

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5
Q

What is Von Willebrand’s disease?

A

vWF deficiency

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6
Q

Von Willebrand’s disease is more commonly diagnosed in (men / women).

Why?

A

women

menorrhagia presents early

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7
Q

Which vasculitis, seen in children, causes abnormal bleeding?

A

HSP

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8
Q

Where should you look for a purpuric rash first?

Why?

A

Lower limbs

Oncotic pressure (gravity) pushes blood out of capillaries into compartments of lower limb

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9
Q

Where should you look for haemorrhages in a patient with abnormal bleeding?

A

Skin

Mouth

Eyes

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10
Q

What are some acquired causes of thrombocytopaenia?

A

Bone marrow pathology e.g malignancy

DIC (widespread clotting uses up all your platelets and clotting factors)

Autoimmune disease (autoimmune thrombocytopaenic purpura)

Renal failure (uraemia causes platelet destruction)

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11
Q

What are autoimmune diseases causing

a) vessel dysfunction
b) platelet destruction

causing abnormal bleeding?

A

a) HSP (vasculitis)

b) ATP

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12
Q

Why can hypersplenism cause platelet destruction?

A

Overactive haemolysis

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13
Q

Which drugs are designed to cause platelet dysfunction?

How do they work?

A

Anti-platelets e.g aspirin, clopidogrel, ticagrelor

Block thromboxane A2

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14
Q

Why does renal failure cause platelet dysfunction?

A

Build up of toxins e.g urea

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15
Q

What is the mode of inheritance of Von Willebrand’s disease?

A

Autosomal dominant

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16
Q

Apart from promoting platelet aggregation, what does vWF do?

A

Carries Factor XIII around

So deficiency also impacts coagulation

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17
Q

Low levels of circulating ___ is the most common reason for failure of haemostasis.

A

platelets

i.e thrombocytopaenia

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18
Q

What is prothrombin also known as?

A

Factor II

just to make things more confusing

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19
Q

In which condition are clotting factors and platelets used up to the point that they are deficient?

A

DIC

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20
Q

Where are clotting factors produced?

A

Liver

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21
Q

Problems with which organ impact coagulation?

A

Liver

where clotting factors are produced

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22
Q

Which vitamin is required to produce clotting factors?

A

Vitamin K

23
Q

Which clotting factors are deficient in

Haemophilia A

Haemophilia B?

A

Haemophilia A - VIII

Haemophilia B - IX

24
Q

Which clotting factors require Vitamin K to be activated?

A

II, VII, IX and X

25
Q

Which clotting factors are inhibited by warfarin therapy?

A

II, VII, IX and X

26
Q

Which clotting time is used to measure warfarin response?

Which clotting factors does warfarin inhibit?

What is required to produce these factors in the liver?

A

PT

II, VII, IX and X

Vitamin K

27
Q

Clotting factors are deficient in ___ failure.

A

liver failure

28
Q

___ _ deficiency causes abnormal bleeding.

A

Vitamin K

required for production of factors II, VII, IX and X in the liver

29
Q

Where is Vitamin K absorbed?

A

Small intestine

30
Q

What is required for Vitamin K to be absorbed in the small intestine?

A

Bile salts

31
Q

Given that Vitamin K is absorbed in the small intestine using bile, what GI conditions can cause Vitamin K deficiency?

A

Malabsorption - Crohn’s disease, resection, bacterial overgrowth

Obstructive jaundice - cholecystitis, pancreatic Ca

32
Q

Which drug acts against Vitamin K?

A

Warfarin

33
Q

Vitamin K can be used to reverse the effects of which drug?

A

Warfarin

34
Q

Why are babies given a Vitamin K injection at birth?

A

To prevent bleeding caused by temporary Vit K deficiency, which is quite common

haemorrhagic disease of the newborn

35
Q

What is DIC?

A

Disseminated intravascular coagulation

Massive, inappropriate activation of haemostasis and fibrinolysis

Using up all your platelets and clotting factors

Which causes deficiencies –> tendency for abnormal bleeding

36
Q

Apart from platelet / clotting factor deficiency, what does massive thrombosis in DIC cause?

A

End organ damage

because all your vessels are occluded

37
Q

Apart from abnormal bleeding, what does DIC cause?

A

End organ damage

because thromboses occlude blood vessels

38
Q

Which enzyme breaks down fibrin?

A

Plasmin

39
Q

What are some causes of DIC?

A

Massive trauma

Sepsis

Obstetric emergencies e.g toxic shock syndrome, placental abruption

Malignancy

Hypovolaemic shock

40
Q

In which type of sepsis is DIC especially common?

A

Meningococcal sepsis

41
Q

How is DIC treated?

A

Treat underlying cause

Replace deficencies

platelet transfusions

plasma transfusions (for clotting factors)

fibrinogen replacement

42
Q

What is the mode of inheritance of haemophilia?

A

X-linked recessive

43
Q

Haemophilia is only expressed in (males / females).

A

males

because it is X-linked recessive

44
Q

Where does bleeding occur in haemophilia?

A

Skin

Mouth

Eyes

Into joints (haemarthrosis)

45
Q

Why does haemarthrosis recur in people with haemophilia?

A

Blood in joint

> Iron in joint (irritant)

> Inflammation

> Recurrent bleeding

46
Q

Haemophilia is a problem with (primary / secondary) haemostasis.

A

secondary haemostasis

47
Q

How is haemophilia treated?

A

Factor VIII / IX injections

48
Q

How regularly are haemophiliacs given clotting factor injections?

A

Every 2 - 3 days

49
Q

Which clotting time is abnormal in haemophilia?

A

APTT

because it measures XIII/IXa action (which are deficient in haemophilia A/B)

50
Q

In which joints does haemarthrosis occur in haemophilia?

A

Ankles

Knees

Elbows

(also: quads, iliopsoas)

51
Q

In multiple factor deficiencies, which clotting times are prolonged?

A

PT

APTT

i.e both of them

52
Q

Name three reasons for multiple factor deficiency.

A

Liver disease

Vitamin K deficiency

DIC

53
Q

Name a reason for single factor deficiency.

A

Haemophilia

Haematology Week 2 2018/19 (7 decks)

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