Bleeding Disorders Flashcards
What is important to ask in history of bleeding disorder
HPC Easy bruising / how long to disappear Epistaxis - normal = <15 minutes Post surgical - dental / tonsils / vaccinations Menorrhagia PPH Post-trauma Any joint pain / limp
PMH
Drug history
Symptoms of anaemia
Systemic Sx - B symptoms / recent infection
FH of bleeding disorder / heavy bleeding / menorrhagia
FH of autoimmune
What is platelet type bleeding / vWF
Mucosal Epistaxis Purpura Petechiae Menorrhagia GI bleeding Retinal haemorrhage Ecchymosis Easily bruising
What is coagulation factor type bleeding
Bleeding into articular joints
Unprovoked muscle haematoma
Brain haemorrhage
How does muscle haematoma present
Swollen hot leg
What is important in Hx to determine if congenital or acquired / type of inheritance
Previous episodes Age of first event Previous challenges with surgery FH Sex
What is haemorrhagic diathesis
Unusualy susceptibility to bleed
What causes
Inhibtion of function of
Platelets
vWF
Coagulation factor
What is haemophilia
X-linked disorder causing coagulation factor deficiency
A = factor 8 (VII) deficiency = most common
B = factor 9 (IX)
What are the clinical features of haemophilia
Risk of severe bleeding in response to minor trauma or spontaneous Haemarthrosis - bleed into joint Muscle haematoma - calf + biceps CNS bleeding Retroperitoneal bleeding Post surgical bleeding
What are the complications of haemophilia
Synovitis Destruction of cartilage Chronic haemophilic arthropathy Neurovascular compression + compartment syndrome Haemorrhagic stroke
How and when does haemophilia present
6 months - 2 years when starting to walk Fall over Stop walking as bleed into knee Bruising Swollen painful leg
How do you Dx haemophilia
Reduced factor 8 or 9
Prolonged APTT
Normal PT
Genetic test but unlikely to have FH
How do you treat haemophilia bleeding
Coagulation factor replacement
DON’t transfuse FFP unless no concentrate factor
DDAVP - releases stored factor 8 + VWf
Tranexamic acid if minor bleed
Recombinant factor if major bleed
Gene therapy
Prophylaxis if severe
When do you give prophylaxis factor concentrate
Severe haemophilia to keep level of factor >2% so don’t bleed spontaneously
Contine for 45 weeks of the year
How do you manage haemophilia
Splints Physio ANalgesia Synovectomy Joint replacement Give vaccines SC instead of IM
What are the complications of treatment
Viral infection - HIV, HBV, HCV, CJD
Development of inhibitors to factor
What are the CI of DDAVP
Elderly as stroke / MI risk
<3 as hyponatraemia / fits
What does severity depend on
Amount of residual coagulation factor in the circulation
What is severe
<1%
Will bleeding spontaneously into muscles and joints every few weeks
What is moderate
1-5%
DONT bleed spontaneous
What is mild
5-30%
Don’t come to harm but may need procedures in place for surgical procedures
What is vWF
AD defect in vWF leading to platelet type bleeding (factor VIII in clotting cascade)
What are the types of vWF
Type 1 = quantitative - don’t make enough
Type 2 = qualitative - abnormal function
Type 3 = severe complete deficiency RARE
How do you Dx and Rx and what should you check
What vaccine
Dx = low levels of vWF in blood
Check blood group as O blood has low levels of vWF
Rx
Tranexamic acid if mild e.g. menorrhagia
DDAVP raises level of vWF by stimulating release = 1st line
vWF concentrate / factor VII for minority if severe
Immunisation against hep A and B
Iron if deficient
