bleeding disorders Flashcards

1
Q

what are the causes of bleeding?

A
  • vascular disorders
  • platelet disorders (thrombocytopenia, defective function)
  • defective coagulation (inherited, acquired)
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2
Q

what alters the pattern of bleeding?

A
  • vascular and platelet causes, bleeding into mucous membranes and skin
  • coagulation disorders, bleeding into joints and soft tissues
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3
Q

what is vascular bleeding?

A

problems with vessel wall

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4
Q

what are the causes of vascular bleeding?

A
  • inherited: hereditary haemorrhagic telangiectasia, ehlers-danlos syndrome
  • acquired: scurvy, steroids, senile
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5
Q

what is thrombocytopenia?

A

low platelets (<150)

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6
Q

when do symptoms of thrombocytopenia occur and what are the symptoms?

A

when platelets are <10 and the syptoms are epistaxis, GI bleeds, menorrhagia, bruising

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7
Q

what causes thrombocytopenia?

A
  • inherited causes (rare)

- acquired causes (common), eg: ITP, drug-related, DIC

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8
Q

what are the treatment options if bleeding or platelets <20?

A

1) steroids and/or intravenous immunoglobulins
2) thromboitein agonists
3) immunosuppression
4) splenectomy

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9
Q

what causes coagulation disorders?

A
  • due to defect in the coagulation cascade
  • inherited causes (rare)
  • acquired causes (common)
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10
Q

what are the different tests of coagulation?

A
  • APTT = activated partial thromboplastin time
  • PT = prothrombin time
  • TT = thrombin time
  • fibrinogen level - clotting factor assay
  • bleeding time (no longer done)
  • D-dimers = breakdown products of fibrin clot
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11
Q

what is haemophilia A?

A

deficiency of factor VIII

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12
Q

what is haemophilia B?

A

deficiency of factor IX (also known as christmas disease)

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13
Q

what type of diseases are haemophilia?

A

X-linked so only affect males

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14
Q

what are clinical features of haemophilia?

A
  • spontaneous bleeding into joints and muscles
  • unexpected post-operative bleeding
  • chronic debilitating joint disease
  • family history in majority of cases
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15
Q

how do you diagnose haemophilia?

A
  • prolonged APTT (test factors VIII, IX, XI, XII in the intrinsic pathway)
  • normal PT (test factors II, V, VII, X) in the extrinsic pathway
  • low factor VIII or IX levels (<1% = severe, 1-5% = moderate and >5% = mild haemophilia)
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16
Q

what are the treatments of haemophilia?

A
  • historic treatments - fractionated human plasma
  • HIV and Hepatitis C infection (and risk of vCJD)
  • current treatment - infusions of recombinants factor VIII or factor IX to 50-100% of normal
  • prophylactic treatment has revolutionised management of haemophilia patients
17
Q

what type of disease is von Willebrand disease?

A

autosomal dominant

18
Q

what is von Willebrant factor?

A

large multimeric protein that carries factor VIII in the blood and it binds platelets to endothelial collagen

19
Q

what is von Willebrand disease?

A

deficiency of von Willebrand factor and FVIII in plasma

20
Q

what are the symptoms of von Willebrand disease?

A
  • mucocutaneous bleeds
  • nosebleeds
  • menorrhagia
21
Q

what are the 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
22
Q

how do you treat von Willebrand disease?

A

treatment with desmopressin, anti-fibrinoytics and plasma products

23
Q

what stimulates release of vWF from Weibel-Palade sotrage bodies from endothelial cells?

A

DDAVP

24
Q

what are acquired disorders of coagulation?

A

non-inherited causes of increased bleeding

25
Q

what are the acquired disorders of coagulation?

A
  • liver disease
  • vitamin K deficiency
  • disseminated intravascular coagulation (DIC)
26
Q

how does the acquired disorders of coagulation affect liver disease?

A
  • deficient synthesis of clotting proteins

- impaired platelet function and fibrinolysis

27
Q

how does the acquired disorders of coagulation affect vitamin k deficiency?

A
  • infants who do not receive vitamin k at birth

- malabsorption due to jaundice

28
Q

how does the acquired disorders of coagulation affect disseminated intravascular coagulation (DIC)?

A
  • release of pro-coagulant material into circulation
  • results in consumption of clotting factors
  • causes both bleeding and thrombosis to occur
29
Q

what is meningococcal DIC?

A
  • prolonged PT, APTT, TT

- low fibrinogen, low platelets

30
Q

how do you know if someone has meningococcal DIC?

A

they have raised D-dimers or FDPs

31
Q

what causes DIC?

A

cancer, sepsis, obstetric diasters

32
Q

what is latrogenic?

A

overdose of anti-coagulant drugs

33
Q

what are the types of anticoagulants?

A
  • heparin = used to treat MIs, PEs, DVTs
  • warfarin = used to treat PEs, DVTs, AF, prosthetic valves
  • DOACs = direct oral anti-coagulants (direct thrombin inhibitors, factor Xa inhibitors)
34
Q

what is vitamin K needed for?

A

required for carboxylation of factors II, VII, IX, X

35
Q

what inhibits vitamin K?

A

warfain

36
Q

what causes vitamin k deficiency?

A
  • malabsorption of vitamin k
  • biliary obstruction
  • haemorrhagic disease of the newborn