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Y3 - Haem > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What are the bleeding disorders?

A

Vascular disorders
Platelet disorders
Von Willebrand disease
Haemophilia
Liver disease
Vitamin K deficiency
Disseminated intravascular coagulation

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2
Q

What are the vascular disorders that cause bleeding problems, and why do they cause them?

A

Why - failure of primary haemostasis
Hereditary:
- Ehlers Danlos
- Marfan’s
- Hereditary haemorrhagic telangiectasia
Acquired
- Senile purpura
- Bruising syndromes
- Vasculitis (Henoch-Shonlein purpura)
- Autoimmune (SLE, RA)
- Meningococcal sepsis

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3
Q

What are the signs of failure of primary haemostasis?

A

Purpura
Mucosal bleeding (GI, menorrhagia, dental procedures, epistaxis)
Easy bruising

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4
Q

What is hereditary haemorrhagic telangiectasia?

A

Autosomal dominant condition causing telangiectasia throughout the body

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5
Q

What is the presentation of hereditary haemorrhagic telangiectasia?

A

Small purpura and telangiectasia on lips, finger tips, nose
Epistaxis
Upper GI bleed

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6
Q

What is the presentation of scurvy?

A

Gingival bleeding and inflammation

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7
Q

What is the presentation of senile purpura and steroid use?

A

Painless bleeding

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8
Q

What causes a bleeding disorder in senile purpura and steroid use?

A

Skin atrophy

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9
Q

What is the presentation of bruising syndrome?

A

Tingling in arms and legs, followed by painful and easy bruising

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10
Q

What are the types of causes of platelet disorders?

A

Decreased production of platelets

Increased use or destruction of platelets

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11
Q

What are the causes of decreased production of platelets?

A 
B12 deficiency
Infection and sepsis
Bone marrow suppression:
- Myelofibrosis
- Myelodysplasia
- Aplastic anaemia
- Leukemia, lymphoma, myeloma, metastases
- Drugs: chemotherapy, radiotherapy, methotrexate
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12
Q

What are the causes of increased use or destruction of platelets?

A

Immune thrombocytopenia purpura
Thrombotic thrombocytuc purpura
Haemolytic uraemia purpura
Disseminated intravascular coagulation
Autoimmune: SLE, anti-phospholipid
Hypersplenism: congestive heart failure, cirrhosis, EBV, malaria, lymphoma, leukaemia
Drugs: heparin, antibiotics (penicillin), sulphonamides, furosemide, digoxin, valproate

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13
Q

What is the presentation of platelet disorders?

A

Symptoms of primary haemostasis defect

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14
Q

What is immune thrombocytopenia purpura?

A

Autoimmune condition of antibody destruction of platelets

Presents differently in children and adults

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15
Q

What is the presentation of immune thrombocytopenia purpura in children?

A

Ages 2-6
Acute onset mild mucosal bleeding and purpura
May be history of recent viral infection

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16
Q

What is the management of immune thrombocytopenia purpura in children?

A

None needed - usually self-limiting

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17
Q

What is the presentation of immune thrombocytopenia purpura in adults?

A

Less acute onset - fluctuating and variable course

Signs of primary haemostasis of variable severity

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18
Q

What is the management of immune thrombocytopenia purpura in adults?

A

Only given if severe
First line: steroids (pred)
Second line: splenectomy
Third line: immunosuppression (azathioprine)

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19
Q

What do blood tests show in immune thrombocytopenia purpura?

A

Thrombocytopenia

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20
Q

What other investigation may be done in immune thrombocytopenia purpura, what would it show, and when would it be done?

A

Bone marrow biopsy
Shows increased megakaryocytes
Only done if there are unusual findings clinically or on blood film

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21
Q

What is thrombotic thrombocytopenia purpura?

A

A haematological emergency where platelet consumption leads to profound thrombocytopenia

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22
Q

What are causes of thrombotic thrombocytopenia purpura?

A 
Hereditary - genetic mutation in protein responsible for breaking down vWF
Pregnancy
Oral contraception
SLE
Infection - e.g. HIV
Drugs - e.g. ticlopidine, clopidogrel
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23
Q

What is the presentation of thrombotic thrombocytopenia purpura?

A 
Purpura
Fever
Mucosal bleeding
Fluctuating cerebral dysfunction - drowsiness, confusion, seizures, hemiparesis
Microangiopathic haemolytic anaemia
AKI with haematuria and purpura
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24
Q

What do investigations show in thrombotic thrombocytopenia purpura?

A

Blood tests:
- thrombocytopenia
low Hb
- high LDH and bilirubin (due to haemolysis)
Coagulation screen normal
Blood film: schistocytes (red cell fragmentation)

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25
Q

What is the management of thrombotic thrombocytopenia purpura?

A

1st line: Plasma exchange (plasmapheresis) plus steroids
2nd line: immunosuppression with rituximab
3rd line: splenectomy
In acquired TTP following resolution of acute episode: long term aspirin

26
Q

What are causes of abnormal platelets?

A 
Drugs: anti-platelets, NSAIDs
Renal disease
Liver disease
Paraprotein
Myeloprroliferative
27
Q

What do blood results show in abnormal platelets?

A

Coagulation normal

Platelet count normal

28
Q

What is von Willebrand disease?

A

Autosomal dominant condition that causes an abnormality in vWF, resulting in reduced function

29
Q

What is the function of von Willlebrand factor?

A

Primary - involved in platelet aggregation

Secondary - stabilises factor VIII to allow factor X complex to form

30
Q

What is the presentation of von Willebrand disease?

A 
Purpura
Easy bruising
Mucosal bleeding
- especially after dental work
- heavy periods in females
31
Q

What do investigations show in von Willebrand disease?

A 
Coagulation:
- increased aPTT
- normal PT
- normal platelets
Serum
- low vWF
- low factor VIII
32
Q

Why do PT and aPTT show the results they do in von Willebrand disease?

A

aPTT is raised because vWF acts on VIII - intrinsic pathway

PT is normal because vWF doesn’t act on tissue factor or factor VII

33
Q

What is the management of mild von Willebrand disease, and what is the action?

A

Desmopressin - promotes release of vWF)

34
Q

What is the management of severe haemorrhage in von Willebrand disease?

A

vWF concentrates

35
Q

What should be avoided in von Willebrand disease?

A

NSAIDs

36
Q

What is haemophilia?

A

Conditions causing failure of fibrin clot formation

37
Q

What is the inheritance pattern of haemophilia?

A

X-linked recessive

38
Q

What does the inheritance pattern of haemophilia mean for parents?

A

Only males are affected, daughters are carriers
Fathers cannot pass it to sons
If a mother is a carrier there is a 50% of a daughter being a carrier, and 50% of a son being affected

39
Q

What are the 2 types of haemophilia, and which clotting factors are affected in each?

A

Haemophilia A - factor VIII

Haemophilia B - factor IX

40
Q

Which type of haemophilia is more common?

A

Haemophilia A

41
Q

When does haemophilia usually first present?

A

In teenagers after trauma or surgery

42
Q

What is the presentation os haemophilia?

A

Large bruising due to bleeding into muscle
Swelling of joints due to bleeding into them
- usually knee affected
- often recurs into the same joint

43
Q

Is there prolonged bleeding in superficial cute in haemophilia?

A

No - primary haemostasis not affected

44
Q

What is the complication of haemophilia?

A

Arthritis due to repeated haemostasis causing inflammation and scar tissue development

45
Q

What do investigations show in haemophilia?

A 
Coagulation:
- isolated increase in aPTT
- normal PT
Serum
- low factor VIII or IX levels
46
Q

What is the management for haemophilia?

A

General: avoid NSAID, IM injections
Active bleeding: factor infusions
Prophylaxis to surgery: factor infusions

47
Q

By which mechanisms does liver disease cause bleeding disorders?

A

Reduced production of clotting factors
Cholestasis resulting in vit K deficiency
Portal hypertension resulting in splenomegaly and increased sequestration of platelets

48
Q

What do investigations show in liver disease?

A

Normal or low platelets
Increased PT and aPTT
Abnormal LFTs

49
Q

What is vitamin K?

A

A fat soluble that carboxylates clotting factors II, VII, IX, X

50
Q

What are causes of deficiency of vitamin K?

A 
Warfarin
Haemorrhagic disease of the newborn
Malabsorption
Poor dietary intake (leafy greens)
Biliary tree obstruction leading to reduced absorption
51
Q

How does warfarin cause vitamin K deficiency?

A

Warfarin inhibits the enzyme that reduces vit K in order for it to be able to carry out it’s function

52
Q

How does haemorrhagic disease of the newborn cause vitamin K deficiency, and how is this prevented?

A

Newborns have little vit D in diet, so no bowel bacteria making vit K
Prevented by IM vit K

53
Q

Where is vitamin K absorbed, and which disorder can impair absorption at this site?

A

Upper small intestine

Crohn’s disease

54
Q

What do investigations show in vitamin K deficiency?

A

Increased PT and aPTT

The Increase in PT is more pronounced than the increase in aPTT

55
Q

What is disseminated intravascular coagulation?

A

Excessive and inappropriate activation of the haemostatic system

56
Q

What is the pathophysiology of DIC?

A

Complex coagulopathy with both thrombosis and coagulation defects
Develops due to widespread release of procoagulant material, resulting in thrombosis and utilisation of clotting factors
To combat thrombosis fibrinolysis is activated, and clots are broken down
Haemorrhage occurs due to using up of platelets and clotting factors
Accumulation of fibrin degradation productions (D-dimers)

57
Q

What are causes of DIC?

A

Sepsis
Trauma
Burns
Surgery
ABO mismatch causing haemolytic transfusion reactions
Obstetric complications - pre-eclampsia, placental abruption

58
Q

What is the presentation of DIC?

A

Thrombosis using multi-organ failure
Large, widespread bruising
Oozing of blood from mouth, nose, cannulation site

59
Q

What do investigations show in DIC?

A

Raised D-dimers
Low platelets
PT and aPTT prolonged

60
Q

What is the management of DIC?

A 
Treat underlying cause
To treat clotting deficits:
- platelet transfusion
- fresh frozen plasma
- cryoprecipitate (replaces fibrinogen, give after FFP)
To treat active bleeding:
- red cell transfusion

Y3 - Haem (9 decks)

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