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103 theme 2 > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

what are causes of bleeding

A

Vascular disorders
Platelet disorders
Defective coagulation

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2
Q

what are types of platelet disorders

A

Thrombocytopenia

Defective function

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3
Q

what are types of defective coagulation

A

Inherited

Acquired

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4
Q

what pattern of bleeding is caused by vascular and platelet disorders

A

Bleeding into mucous membranes and skin

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5
Q

what pattern of bleeding is caused by coagulation disorders

A

Bleeding into joints and soft tissues

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6
Q

what are inherited issues with vessel walls

A

Hereditary haemorrhagic telangiectasia

Ehlers-Danlos syndrome

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7
Q

what are acquired issues with vessel walls

A

Scurvy, steroids, senile

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8
Q

what is Hereditary haemorrhagic telangiectasia

A

Also known as Osler-Weber-Rendu

haemorrhage spots on tongue lips (mucous membranes?)

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9
Q

what is the normal range of platelets

A

150-400 x 109/L

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10
Q

what is the range of platelets to thrombocytopenia

A

<150

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11
Q

what are symptoms when platelets are less than 10

A

Epistaxis, GI bleeds, menorrhagia, bruising

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12
Q

what are other causes of thrombocytopenia

A 
Inherited causes (rare) or Acquired causes (common)
eg: ITP, drug-related, DIC
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13
Q

what is ITP

A

immune thrombocytopenia

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14
Q

What are treatment options if bleeding or platelets <20

A

steroids and/or intravenous immunoglobulins
thromboietin agonists e.g., romiplostim
immunosuppression e.g. rituximab
splenectomy

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15
Q

what is a key sign of disorders of platelet function

A

Platelet count – typically normal

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16
Q

what are types of disorders of platelet function

A

Inherited, many rare diagnoses,
e.g., Glanzmann’s thrombasthenia

Acquired, Drugs – much more common
e.g., Aspirin, NSAIDs

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17
Q

what causes disorders of coagulation

A

Due to a defect in the coagulation cascade

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18
Q

what are types of coagulation disorders

A

Inherited causes (rare)

Acquired causes (common)

19
Q

what clotting factors are TF, Prothrombin, Thrombin, Fibrinogen

A 
TF  III
Prothrombin II
Thrombin IIa
Fibrinogen I
Prothrombinase X,V,Ca2+,PL
20
Q

What are tests of coagulation

A

APTT
PT
TT

21
Q

What is APTT

A

activated partial thromboplastin time to assesses the intrinsic pathway

22
Q

What is PT

A

prothrombin time to assesses the extrinsic pathway

23
Q

what is TT

A

thrombin time to assesses terminal common pathway

24
Q

what are other tests of coagulation

A

Fibrinogen level
Clotting factor assays (normal level 100%)
Bleeding time (time to stop bleeding) - no longer done
D-dimers = breakdown products of fibrin clot

25
Q

what are the types of haemophilia

A

Haemophilia A – deficiency of factor VIII
Haemophilia B – deficiency of factor IX
(also known as Christmas disease)

26
Q

what are the 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)

27
Q

how is haemophilia diagnosed

A

Prolonged APTT
tests factors VIII, IX, XI, XII in the intrinsic pathway

Normal PT
tests factors II, V, VII, X in the extrinsic pathway

Low factor VIII or IX levels

28
Q

what is considered low for factor VIII or IX in haemophilia

A

<1% = severe; 1-5% = moderate; >5% = mild haemophilia

29
Q

what are historic treatments for haemophilia

A

Historic treatments – fractionated human plasma but risk of HIV and Hepatitis C infection (and risk of vCJD)

30
Q

what are current treatments for haemophilia

A

Current treatment – infusions of recombinant factor VIII or factor IX to 50-100% of normal

Prophylactic treatment has revolutionised management of haemophilia patients

31
Q

what is von willebrand disease

A

Autosomal dominant
vWD = deficiency of vWF and FVIII in plasma

Mucocutaneous bleeds, nosebleeds, menorrhagia

32
Q

what is von willebrand factor

A

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

33
Q

what are tests for diagnosing vWD

A 
Prolonged APTT
Normal PT
Low vWF antigen level and/or vWF function
Low factor VIII level
Prolonged bleeding time
Defective platelet function
34
Q

how can vWD be treated

A

Treatment with desmopressin (DDAVP), anti-fibrinolytics and plasma products

35
Q

what does DDVAP do for vWD

A

DDAVP stimulates release of vWF from Weibel-Palade storage bodies from endothelial cells

36
Q

what are acquired disorders for coagulation

A

These are non-inherited causes of increased bleeding

Liver disease, bit K deficiency, disseminated intravascular coagulation (DIC)

37
Q

How is liver disease an acquired disorder of coagulation

A

Deficient synthesis of clotting proteins

Impaired platelet function and fibrinolysis

38
Q

How is vit K deficiency an acquired disorder of coagulation

A

Infants who do not receive vitamin K at birth

Malabsorption due to jaundice

39
Q

How is DIC an acquired disorder of coagulation

A

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

40
Q

What are anti coagulant drugs

A

Heparin – used to treat MIs, PEs, DVTs
Warfarin – used to treat PEs, DVTs, AF, prosthetic valves
DOACs = direct oral anti-coagulants
Direct thombin inhibitors (dabigatran, argatroban)
Factor Xa inhibitors (rivaroxaban, apixaban)

41
Q

what causes a vitamin k deficiency

A

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

42
Q

what is vitamin k used for

A

Vitamin K is required for gamma-carboxylation of factors II, VII, IX, X

Inhibited by warfarin (monitored using the INR)

43
Q

what is Haemoarthritis

A

bleeding in the joints

103 theme 2 (7 decks)

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