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Haematology > disorders of haemostasis > Flashcards

disorders of haemostasis Flashcards

1
Q

what are bleeding disorders?

A

Deficiency in coagulation factors that cause a tendency to bleed excessively

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2
Q

what are the 2 types of bleeding disorders?

A

Hereditary

Acquired

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3
Q

what are 2 possible reasons for bleeding disorders?

A

Low levels of coagulation factors

Disfunctional coagulation factors

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4
Q

what are the hereditary bleeding disorders?

A

Haemophilia A
Haemophilia B
Haemophilia C
Von Willebrands disease

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5
Q

what is haemophilia A?

A

Deficiency of factor VIII

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6
Q

when does haemophilia A occur?

A

Absence or low levels of F VIII

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7
Q

what are the symptoms of haemophilia A?

A 
Onset of symptoms in infancy
Profuse bleeding following circumcision
Excessive bruising when start to become active
Muscle haematomas
Haemarthroses
Bleeds into joints
Bleeds into internal organs
Post-op and post-trauma haemorrhage can be life-threatening
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8
Q

is haemophilia A more common in males or females?

A

males - X linked disorder

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9
Q

what causes Haemophilia B?

A

Absence or low levels of F IX

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10
Q

how is the difference between haemophilia A and haemophilia B distinguished?

A

Distinguished only by factor IX assay

– Measure of factor IX levels

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11
Q

what causes haemophilia C?

A

Deficiency of FXI

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12
Q

what does treatment of haemophilia include?

A
  • In specialised haemophilia centres
  • Treated with prophylactic factor replacement therapy
  • Reduced crippling haemarthroses
  • Additional treatment to cover surgery, trauma and spontaneous bleeds
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13
Q

what is included in the laboratory diagnosis of haemophilia A, B and C?

A
  • Activated Partial Thromboplastin Time test (APTT)
    • Measures the intrinsic coagulation pathway
  • – Extended (XII,XI, IX and VIII deficiency)
  • Prothrombin time (PT)
    • Measures extrinsic coagulation pathway
  • – Normal
  • Assay to measure level of coagulation factor
    • Reduced
  • Platelet function
    • Normal
  • Bleeding time
    • Normal
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14
Q

which is the most common hereditary bleeding disease?

A

Von Willebrand’s disease?

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15
Q

why is Von Willebrand’s disease termed pseudo-haemophilia?

A

no joint bleeds.

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16
Q

what are the 3 subtypes of von Willebrand’s disease?

A

Type I - Mild reduction in VWF

Type II - Functionally abnormal VWF

Type III - No VWF, most severe form

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17
Q

what is the role of von Willebrand factor in the coagulation cascade?

A
  • promotes platelet adhesion

- carrier molecular for factor VIII

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18
Q

what is the laboratory diagnosis of vWD?

A
  • Abnormal platelet function tests
  • Defective platelet aggregation
  • Low FVIII levels
  • APPT prolonged (Intrinsic pathway)
  • vWF levels low
  • Low platelet count in type 2
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19
Q

what is the treatment for type I von Willebrand disease?

A

DDAVP (vasopressin)

– Releases vWF from endothelial cells

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20
Q

what is the treatment for type II and III von Willebrand’s disease?

A
  • Plasma derived FVIII/vWF concentrates

- Recombinant vWF in phase II clinical trials

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21
Q

what are other clotting factor deficiencies?

A
  • fibrinogen, FV, FVII

- Prothrombin, FXIII

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22
Q

what causes acquired bleeding disorders?

A
  • production problems

- consumption problems

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23
Q

what are examples of production problems that lead to acquired bleeding disorders?

A
  • Vitamin K deficiency

- Liver disease

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24
Q

what are examples of consumption problems that lead to acquired bleeding disorders?

A
  • disseminated intravascular coagulation (DIC)
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25
Q

which factors are impacted by vitamin K deficiency?

A

decreased synthesis of factors II VII IX X

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26
Q

what are the diagnostic test results of vitamin K deficient acquired bleeding disorder?

A

Abnormal PT and APTT

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27
Q

how does liver disease lead to an acquired bleeding disorder?

A

impaired absorption of vitamin K

28
Q

as well as APPT and PT being abnormal what else is affected due to liver disease?

A

intrinsic and extrinsic pathway

29
Q

what is DIC secondary to?

A

Sepsis
Malignancy
Acute myeloid leukaemia
Snake venom

30
Q

what does DIC lead to?

A
  • Deposition of fibrin in the microcirculation
    • amputation
  • Haemorrhage due to consumption of coagulation factors and platelets
31
Q

what causes DIC?

A

Increase of TF expression

32
Q

what are acquired inhibitors of coagulation?

A

Inhibitors of factor VIII

33
Q

what are the hereditary disorders of platelet function?

A
  • Glanzmann’s thrombasthenia
  • Bernard Soulier syndrome
  • von Willebrand disease
34
Q

what occurs during Glanzmanns disease?

A

failure of primary platelet aggregation

35
Q

what causes Glanzmanns disease?

A

Deficiency in membrane protein GPIIbIIIa

36
Q

what is seen in Glanzmann’s disease?

A

Clinically severe platelet function defect

37
Q

what does Clinically severe platelet function defect lead to?

A 
Epistaxis
Purpura
Bruising
Gum bleeding
Menorrhagia
Bleeding after surgery
38
Q

what causes Bernard Soulier syndrome?

A

Absent or decreased expression of GPIb-V-IX complex on platelet surface

39
Q

what occurs in Bernard Soulier syndrome?

A
  • Defective binding to vWF
  • Defective adherence to exposed subendothelial connective tissue
  • Variable degree of thrombocytopenia
40
Q

what is seen in Bernard Soulier syndrome?

A

large/giant platelets

41
Q

what are the acquired disorders of platelets that are due to production?

A

Leukaemia
Post viral
Immunosuppression

42
Q

what can shorten the lifespan of platelets and lead to acquired disorders?

A

malaria

bleeding

43
Q

what impacts the function of platelets and lead to acquired disorders?

A

drugs

44
Q

what are the drugs that affect platelet function?

A 
Aspirin/NSAIDs
Caffeine
Antimicrobials
Cardiovascular agents
Anticoagulants
Chemotherapy drugs
45
Q

what is thrombosis?

A

Formation of a solid mass, a thrombus, in the lumen of a blood vessel during life.

46
Q

what is a thrombus formed from?

A

Formed from blood constituents

  • Platelets
  • Fibrin
47
Q

what are the 2 types of thrombosis?

A

Venous thrombosis-occurs in veins

Arterial thrombosis-occurs in arteries

48
Q

what are thrombi associated with?

A
  • Myocardial infarction
  • Cerebral vascular disease
  • Deep vein thrombosis (DVT)
  • Pulmonary embolism (PE)
49
Q

what is the pathogenesis of arterial thrombosis?

A
  • Atherosclerosis of vessel wall
  • Injury to vessel wall
  • Expose blood to subendothelial collagen and tissue factor
  • Formation of platelet nidus
  • Platelets adhere and aggregate
  • Block artery
50
Q

what are the risk factors of arterial thrombosis?

A
  • Family history
  • Male
  • Raised lipids
  • Raised blood pressure
  • Diabetes
  • Smoking
51
Q

what is the pathogenesis of venous thrombosis?

A
  • Coagulability of blood
  • Slowing down of blood flow
  • Coagulation of blood at site of initiation of thrombus
52
Q

what are the risk factors for venous thrombosis?

A
  • Factor V leiden gene mutation
  • Prothrombin gene mutation
  • Protein S and C deficiency
  • ABO blood group
  • Raised levels of factor VIII, fibrinogen and homocysteine
  • Lupus anticoagulant
  • Oral contraception
  • Pregnancy
  • Trauma
  • Malignancy
  • Obesity
  • Age
  • Varicose veins
  • Immobility
53
Q

when is hereditary thrombophilia suspected?

A
  • Young people with spontaneous thrombosis
  • Recurrent DVT
  • Unusual site of thrombosis
54
Q

what is the most common inherited cause of venous thrombosis?

A

Factor V leiden mutation

55
Q

what happens in Factor V leiden mutation?

A

Arginine replaced with glutamine position 560 on factor V gene

56
Q

what does antithrombin deficiency lead to?

A

Recurrent venous thrombosis in early adult life

57
Q

how is protein C activated?

A

by thrombin

58
Q

what is the function of protein C?

A

Cleaves activated factor V and VIII

– Inactivates them

59
Q

what is protein S?

A

Cofactor for protein C

60
Q

what does prothrombin gene mutation lead to?

A

Causes increased levels of prothrombin in circulation

61
Q

what happens in the prothrombin gene mutation?

A

guanine to adenine at position 20210

62
Q

which blood groups have a higher risk of thrombosis?

A

Group A, AB and B

63
Q

what is the treatment of thrombosis?

A
  • Heparin
  • Oral anticoagulants
    • Warfarin
64
Q

what are some new drug types used to treat thrombosis?

A
  • FXa inhibitors
  • Direct thrombin inhibitors
  • Antiplatelet drugs
65
Q

what are example of FXa inhibitor drugs?

A

Fondaparinux

Rivaroxaban

66
Q

what are examples of direct thrombin inhibitor drugs?

A

bivalirudin

dabigatran

67
Q

what are examples of antiplatelet drugs?

A

Clopidogrel

Aspirin

Haematology (7 decks)

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