Theme 2: Lecture 6 - Bleeding disorders Flashcards

1
Q

What causes bleeding disorders

A
  • Vascular disorders
  • Platelet disorders
  • Defective coagulation
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2
Q

What pattern of bleeding is seen in vascular and platelet disorders

A

bleeding into mucous membranes and skin

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3
Q

What pattern of bleeding is seen in coagulation disorders

A

Bleeding into joints and soft tissues

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4
Q

haemarthrosis

A

Bleeding into the joints

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5
Q

What causes vascular bleeding

A

problems with the vessel wall

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6
Q

Name 2 inherited vascular bleeding disorders

A
  • Hereditary haemorrhagic telangiectasia

- Ehlers-Danlos syndrome

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7
Q

Name 3 acquired vascular bleeding disorders

A
  • Scurvy
  • Steroids
  • Senile
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8
Q

What is hereditary haemorrhagic telangiectasia also known as

A

Osler-Weber-Rendu

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9
Q

What are 2 platelet disorders

A
  • Thrombocytopenia

- Defective function

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10
Q

Normal platelet range

A

150-400 x 10^9/L

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11
Q

Thrombocytopenia platelet range

A

<150 /L

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12
Q

When are there symptoms of thrombocytopenia

A

Platelets < 10/L

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13
Q

Symptoms of thrombocytopenia

A
  • Epistaxis
  • GI bleeds
  • Menorrhagia
  • bruising
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14
Q

Epistaxis

A

Nose bleeds

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15
Q

acquired causes of thrombocytopenia

A
  • Immune thrombocytopenia purapura (ITP)
  • Drug related
  • Disseminated intravascular coagulation (DIC)
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16
Q

DIC

A
  • Disseminated intravascular coagulation
  • abnormal blood clotting throughout the body’s blood vessels
  • caused by another disease or condition that makes the body’s normal blood clotting process become overactive.
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17
Q

ITP

A
  • Immune thrombocytopenia purapura

- Autoimmune condition where body generates antibodies against own platelets

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18
Q

Treatment options for ITP if bleeding or platelets are below 20/L

A
  • steroids and/or intravenous immunoglobulins
  • thromboietin agonists
  • immunosuppression
  • splenectomy
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19
Q

Romiplostim

A

a thromboietin agonist

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20
Q

Rituximab

A

an immunosuppression drug

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21
Q

thromboietin

A

Hormone that stimulates production of platelets

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22
Q

Name an inherited platelet disorder

A

Glanzmann’s thrombasthenia

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23
Q

What causes acquired platelet disorders

A

Drugs e.g.

  • Aspirin
  • NSAIDs
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24
Q

NSAIDs

A

Nonsteroidal anti-inflammatory drugs

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25
Q

What are disorders of coagulation due to

A

defect in the coagulation cascade

26
Q

APTT

A

Activated partial thromboplastin time

27
Q

What does the APTT assess

A
  • The intrinsic pathway

- Factors VIII, IX, XI, XII

28
Q

PT

A

Prothrombin time

29
Q

What does the PT assess

A
  • The extrinsic pathway

- Factors II, V, VII, X

30
Q

TT

A

Thrombin time

31
Q

What does the TT assess

A

The common terminal pathway

32
Q

What are the tests for coagulation

A
  • APTT = activated partial thromboplastin time
  • PT = prothrombin time
  • TT = thrombin time
  • Fibrinogen level
  • Clotting factor assays (normal level 100%)
  • Bleeding time (time to stop bleeding) - no longer done
  • D-dimers = breakdown products of fibrin clot
33
Q

What are the inherited coagulation disorders

A
  • Haemophilia A and B

- von Willebrand disease

34
Q

What causes Haemophilia A

A

Factor VIII deficiency

35
Q

What causes Haemophilia B

A

Factor IX deficiency

36
Q

Clinical features of Haemophilia

A
  • Spontaneous bleeding into joints and muscle
  • Unexpected post-operative bleeding
  • Chronic debilitating joint disease
  • Family history in majority of cases
  • X-linked recessive
37
Q

Haematoma

A

An accumulation of blood in the tissues

38
Q

Diagnosis for haemophilia

A

-Prolonged APTT
-Normal PT
-Low factor VIII or IX levels
<1% = severe; 1-5% = moderate; >5% = mild haemophilia

39
Q

Treatment for haemophilia

A
  • infusions of recombinant factor VIII or factor IX to 50-100% of normal
  • Prophylactic treatment has revolutionised management of haemophilia patients
40
Q

Tests for diagnosing von Willebrand disease

A
  • Prolonged APTT
  • Normal PT
  • Low vWF antigen level and/or vWF function
  • Low factor VIII level
  • Prolonged bleeding time
  • Defective platelet function
41
Q

Treatment of von Willebrand disease

A
  • Desmopressin AKA DDAVP
  • anti-fibrinolytics
  • Plasma products
42
Q

What does desmopressin (DDAVP) do

A

stimulates release of von Willebrand factor from Weibel-Palade storage bodies from endothelial cells

43
Q

Weibel-Palade bodies

A

small storage granules in the endothelial cells of the blood vessels and heart

44
Q

von Willebrand disease

A
  • Autosomal dominant disease

- Deficiency of von Willebrand factor and factor VIII in the plasma

45
Q

Symptoms of von Willebrand disease

A
  • Mucocutaneous bleeds
  • nosebleeds
  • menorrhagia
46
Q

What is von Willebrand factor

A
  • large multimeric protein that carries factor VIII in the blood
  • vWF also binds platelets to endothelial collagen
47
Q

What are some acquired causes of coagulation disorders

A
  • Liver disease
  • Vitamin K deficiency
  • Disseminated Intravascular Coagulation (DIC)
48
Q

How does liver disease cause coagulation disorders

A
  • Deficient synthesis of clotting proteins

- Impaired platelet function and fibrinolysis

49
Q

How does DIC cause coagulation disorders

A
  • Release of pro-coagulant material into circulation
  • Results in consumption of clotting factors
  • Causes both bleeding and thrombosis to occur
50
Q

What is seen in meningococcal DIC

A
  • Prolonged PT, APTT, TT
  • Low fibrinogen, low platelets
  • Raised D-dimers or FDPs (fibrin degradation products)
51
Q

Anti-coagulation drugs

A
  • Heparin
  • Warfarin
  • DOACs = direct oral anti-coagulants
  • Direct thombin inhibitors
  • Factor Xa inhibitors
52
Q

What is heparin used to treat

A

MIs, PEs, DVTs

53
Q

What is warfarin used to treat

A

PEs, DVTs, AF, prosthetic valves

54
Q

DOACs

A

direct oral anti coagulants

55
Q

name 2 direct thrombin inhibitors

A
  • dabigatran

- argatroban

56
Q

Name 2 factor Xa inhibitors

A
  • rivaroxaban

- apixaban

57
Q

What is vitamin K required for

A

for gamma-carboxylation of factors II, VII, IX, X

58
Q

What is vitamin K inhibited by

A

Warfarin

59
Q

INR

A
  • International normalized ratio

- Standard measurement that works out how long it takes blood to clot

60
Q

What causes vitamin K deficiency

A
  • Malabsorption of vitamin K
  • Biliary obstruction (jaundice)
  • Haemorrhagic disease of the newborn (give 1mg at birth)