Bleeding Disorders Flashcards
What 3 groups do bleeding disorders fall under?
Vascular defects
Platelets
Coagulation disorders
S+S:
What may patients notice on their skin?
Mucosal bleeding - where does this occur?
What may happen in women?
Where may they have internal bleeding?
Why is caution taken with surgery or trauma?
Haemarthrosis and haematoma are common in clotting factor deficiencies. What are they?
Easy bruising
Gum
Epistaxis
Menorrhagia
GI
Intracranial
Prolonged bleeding
A condition that occurs as a result of bleeding into a joint cavity.
A haematoma, also spelt haematoma, is localized bleeding outside of blood vessels, due to either disease or trauma including injury or surgery and may involve blood continuing to seep from broken capillaries.
Investigations:
2 basic bloods that should be done?
Clotting studies - aPTT:
- What does it stand for?
- What pathway does it test?
- What is lupus anticoagulant?
- What drug may increase aPTT?
Clotting studies - PT:
- What pathway does it test?
- Why does Vit K deficiency affect this before the PT?
- What type of disease is it raised in?
What is the standardised form for both?
What can be done to look for a specific factor deficiency?
THE COAG SCREEN COULD GIVE YOU FALSE RESULTS SO BE AWARE!
Unfractionated heparin
FCB
U&E
LFTs
Activated partial thromboplastin time
Intrinsic pathway - therefore raised in haemophilia
Its name is a misnomer, as it is actually a prothrombotic antibody. Lupus anticoagulant in living systems cause an increase in inappropriate blood clotting.
Extrinsic pathway
Factor 7 has the shortest half life so it affects the extrinsic pathway first.
Liver disease as it makes prothrombin - normal in haemophilia
INR - international normalised ratio
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Factor assay
HAVE A LOOK AT THE COAG CASCADE
General management:
What drugs should they avoid?
What type of injection should they avoid?
What antifibrinolytic can be given to stabilise clot?
NSAIDs
Anticoagulants
IM
Tranexamic acid
Vascular defects:
What does this mean?
Congenital causes:
- Osler-Weber-Rendu syndrome - what is it?
- Connective tissue disorders (e.g. Ehlers-Danlos Syndrome) - what is it?
Acquired causes:
- What infections can cause this?
- Henoch-Schonien purpure - what is it?
Impaired vasoconstriction due to vascular defects
Hereditary haemorrhagic telangiectasia (HHT) is an inherited genetic disorder that affects the blood vessels. It’s also known as Osler-Weber-Rendu syndrome. People with HHT have some blood vessels that have not developed properly and sometimes cause bleeding, known as arteriovenous malformations (AVMs).
An arteriovenous malformation (AVM) is an abnormal tangle of blood vessels connecting arteries and veins, which disrupts normal blood flow and oxygen circulation.
Ehlers-Danlos syndromes are a group of conditions that cause very flexible joints and stretchy and fragile skin. ---- Meningococcal Measles Dengue
Henoch-Schönlein purpura (HSP) is a rare condition in which blood vessels become inflamed. It typically results in a rash and joint and tummy pain.
Coagulation disorders:
Inherited causes - 2
Acquired:
- What 2 types of haematology drugs can cause it?
- What organ disease can cause it?
- Why does kidney disease affect platelet function?
- What vit is needed for normal coag?
Haemophilia
VWD
Anticoagulants
Antiplatelets
Liver disease
Under normal conditions, ADP and serotonin are secreted to attract more platelets. In renal failure patients, their platelet granules have decreased levels of ADP and serotonin.
Vit K
Haemophilia:
What type of genetic disease is it?
Haemophilia A:
- What clotting factor does it affect?
Haemophilia B:
- What clotting factor does it affect?
What type of bleeding is a big sign of haemophilia?
X-linked mutation, therefore linked to males
Factor 8 VIII
Factor 9 IX
Joint bleeding
Haemophilia:
S+S:
Haemoarthrosis leads to long term arthropathy. What do both of these terms mean?
Haematoma in muscles:
- What symptom will patients complain of?
- What will happen due to increased pressure in the fascia?
- What happens to the nerves due to increased pressure in the fascia?
Hemarthrosis is a condition that occurs as a result of bleeding into a joint cavity. A joint that has recurring hemarthrosis (bleeding episodes) is known as a target joint, which typically means that around four separate bleeds have occurred in the same joint over a 6-month period.
Arthropathy means any disease of the joints.
Severe pain
Compartment syndrome
Nerve palsies
Haemophilia:
Diagnosis:
- Apart from coag testing, what can be other blood test can be done for both types?
Management:
- What type of sport should they avoid?
- What type of injection should be avoided?
- What can be given which would obviously solve the problem?
Factor 8/9 assay - look up - The factor VIII assay is a blood test to measure the activity of factor VIII.
Contact sports - due to injury
IM - use SC instead - look at page 345
IV Recombinant F8/9 concentrate
Haemophilia - Management of minor bleeding:
What can be done conservatively?
Desmopressin:
- How does it work?
- What the maximum number of doses?
Haemophilia - Management of major bleeding:
What is classed as a major bleed?
What can be given to make sure there ie enough F8/9?
What should you not forget to also investigate?
Pressure
Elevation
Increases F8 levels
Max 3 doses
Haemoarthrosis - very bad
IV recombinant F8/9 concentrate
Any family members as well
Von Willebrand Disease:
What does lack of VWF prevent?
What factor is affected?
List bleeding that could happen due to this?
Apart from recombinant factor 8, what else can be given to reduce the risk of bleeding?
Prevents platelet binding
Lowers F8 binding and protection
Factor 8 as the half life is reduced
Mucosal bleeding (e.g. epitaxis, menorrhagia)
GI bleeding
Postpartum bleeding
Post-surgical bleeding
Tranexamic acid
Liver Disease:
What 3 things does the liver do?
Reduced synthesis of clotting factors
Reduced absorption of Vit K
Abnormalities of platelet function
Malabsorption:
What can be done if there is a lack of vit K absorption?
IV Vit K (10 mg)
Causes of prolonged PT and aPTT:
What organ failure would cause this?
What types of meds ….?
What does supratherapeutic warfarin therapy mean?
What diseases may consume clotting factors?
Liver failure
DOAC
Warfarin prolongs PT but it doesn’t prolong aPTT – it may prolong aPTT as well which means it has a super therapeutic affect
DIC
Presentations of bleeding disorders:
What may happen after birth that may suggest a bleeding disorder?
What about childhood?
What about adulthood?
What tool can be sued to assess risk of bleeding?
Prolonged umbilical stump bleed
Subdural haematoma
A lot of bruising
Non-accidental injury
Easy bruising
Menorrhagia
Bleeding tendency - epistaxis
Heavy bleeding after teeth extraction
Bleeding assessment tool
