Bleeding Disorders Flashcards

1
Q

Basic components of Haemostasis

A

primary - platelet plug
secondary - fibrin clot
fibrinolysis
anticoagulant defences

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2
Q

when is secondary Haemostasis used

A

if the platelet plug is not enough and need a more stable clot

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3
Q

how do platelets bind directly to the damaged endothelium/collagen

A

glycoproteins (A4B1 and glycoprotein VI)

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4
Q

how do platelets stick to VWF

A

via glycoprotein Ib

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5
Q

what can go wrong with the formation of a platelet plug

A

Vascular problem
Platelet problems (reduced number - thrombocytopenia, or reduced function)
Von Willebrand Factor

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6
Q

what is the most common cause of thrombocytopenia

A

autoimmune thrombocytopenia

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7
Q

causes of reduced function of platelets

A

often drug adverse effects

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8
Q

Causes of vascular abnormalities

A
Hereditary disorders (rare)
-marfans (collagen problems) 

Acquired

  • vasculitis eg. HSP IgA mediated vasculitis in children
  • scurvy (vit c needed for collagen production)
  • senile purpura (less collagen in walls in old age)
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9
Q

what causes thrombocytopenia

A

hereditary (rare)

Acquired

  • reduced production (bone marrow problems eg. leukaemia/lymphoma)
  • increased destruction (most common cause, immune related)
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10
Q

what comes along with reduced production thrombocytopenia

A

pancytopenia

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11
Q

what are the causes of peripheral platelet destruction

A

Coagulopathy
-anything causing a lot of tissue damage activates both primary and secondary Haemostasis and uses up the platelets

Autoimmune
-anti-bodies against platelets

Hypersplenism
-liver disease = most common cause as blood cant get into liver so it backs up through the portal vein and damages/enlarges the spleen

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12
Q

what does platelet destruction look like on blood film

A

platelets start off big and then get smaller

increased big platelets due to increased destruction?????

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13
Q

what causes platelet function defects

A

Hereditary
-rare

Acquired

  • drugs (aspirin, NSAIDs)
  • renal failure
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14
Q

causes of VWF deficiency

A

Acquired
-autoimmune

Hereditary

  • AD
  • common
  • variable severity (generally mild)
  • present with primary Haemostasis problems (mucosal bleeding, epistaxis, menorrhagia etc)
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15
Q

what is the most common cause of primary haemostatis dysfunction

A

Thrombocytopenia usually acquired

caused by marrow failure or peripheral destruction

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16
Q

treatment for peripheral destruction thrombocytopenia

A

treat cause - cant give platelet transfusion because they’ll just get broken down

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17
Q

causes of failure of secondary Haemostasis (fibrin clot formation)

A

Multiple clotting factor deficiencies
(acquired eg. disseminated intravascular coagulation)

single clotting factor deficiency

  • hereditary
  • haemophilia
18
Q

what is disseminated intravascular coagulation

A

clotting factors used up faster than can be made

multiple thrombi in the capillaries

often caused by underlying sepsis

19
Q

Causes of multiple clotting factor deficiency

A
Liver failure 
 (if low albumin check clotting factors, prolongation of prothrombin time and activated partial prothrombin time) 

Vitamin K deficiency/warfarin therapy

Complex coagulopathy
-disseminated intravascular coagulation

20
Q

which 4 blood clotting factors require vitamin K to work

A

II, VII, IX, X

2,7,9,10 are carboxylated by vit K

21
Q

causes for low vitamin K

A
warfarin 
poor dietary intake 
malabsorption 
obstructive jaundice (bile salts cant get out) 
haemorrhage disease of the newborn
22
Q

where is vitamin K from

A

Diet
Intestinal synthesis

baby’s have vit K deficiency at birth, all given IM vit K at birth to prevent haemorrhage disease of the newborn

23
Q

how is vit K absorbed

A

absorbed in upper intestine

required bile salts for absorption

24
Q

common dietary source of vit K

A

leafy green veg

25
Q

what is warfarin used to treat most commonly

A

Atrial fibrillation

to prevent blood clot breaking off and causing a stroke (bc in AF blood isn’t pumped out the heart properly so pools and forms a clot in LV)

26
Q

what is disseminated intravascular coagulation

A

Excessive inappropriate activation of haemostats system
(primary, secondary and fibrinolysis)

microvascular thrombus formation
-end organ failure

clotting factor consumption and break down

  • bruising
  • purpura
  • generalised bleeding
27
Q

what happens if you use up all your clotting factors

A

prothrombin time and Activated partial thromboplastin time

28
Q

what is increased in vit K deficiency

A

prolonged prothrombin time and activated partial thromboplastin time

29
Q

what is the most sensitive test for liver disease

A

prothrombin time

-because factor VII has the shortest half life so is the first one to go

30
Q

what fibrin degradation products can be measured

A

D-dimers

to show increased breakdown of fibrin clots

used to identify of PE/DVT

31
Q

causes of disseminated intravascular coagulation

A

Sepsis
Obstetric emergency (placental abruption)
Malignancy (adenocarcinoma)
Hypovolaemic shock

32
Q

treatment of DIC

A

treat underlying cause (eg. antibiotics)

Replacement therapy

  • platelet transfusions
  • plasma transfusions (FFP)
  • fibrinogen replacement (cryoprecipitate)
33
Q

what causes an individual clotting factor deficiency

A

Heretidary conditions

34
Q

What is haemophilia

A

X-linked disorder

abnormality prolonged bleeding recurs episodically at one or a few sites on each occasion

no primary Haemostasis problems

35
Q

what is haemophilia A

A

Factor VIII deficiency

36
Q

what is haemophilia B

A

factor IX deficiency

37
Q

presentation of haemophilia

A

bleeding from medium to large blood vessels

milk moderate and severely effected families depending on factor VII/IC level

38
Q

what is prolonged in haemophilia

A

v prolonged activated partial thromboplastin time (VIII/IXa not working at all)

prothrombin time is fine (TF-VIIa is working normally)

39
Q

clinical features of severe haemophilia

A

recurrent haemarthroses

recurrent soft tissue bleeds (bruising in toddlers)

prolonged bleeding after dental extractions, surgery and invasive procedures

40
Q

how are haemophilias managed

A

IV clotting factor given exert second day