bleeding disorders Flashcards
points to cover in bleeding hx
has the patient actually got a bleeding disorder
how severe is the disorder - what does it take to make the patient bleed
pattern of bleeding
congenital/acquired
mode of inheritance
hx of bleeding
bruising
epistaxis
post surgical bleeding - dental surgery, circumcision, tonsillectomy, appendicectomy
menorrhagia (good -ve predictive factor for vW disease)
post-partum haemorrgage
post-trauma
what score can be done for bleeding
BAT score
gives you a quantitative score for assessing bleeding symptoms
severity of bleeding in hx
how appropriate is the bleeding for the situation
level of trauma - is the bleeding expected or far more than normal
any unprovoked bleeding - indicative of severe coagulopathic abnormality
platelet type pattern of bleeding
mucosal epistaxis - doesn't stop or stops and quickly restarts again purpura menorrhagia GI
post-surgical/post-interventional bleeding
coagulation factor pattern of bleeding
articular - esp hinge joints e.g. knee, ankle, elbow muscle haematoma (spontaneous of after minor injury) CNS post intervention/surgery bleeding
classic feature of petechiae
don’t blanch when pressed
red lesions that would blanch would have vessel involvement e.g. telangiectasia, arterioles, spider veins etc
how does haemophilic arthropathy occur
bleeding into the joint
iron released from red cells is taken up by macrophages
inflammatory response
boggy synovitis as a result
substances released prevent the repair of normal cartilage in the joint
how to determine is bleeding is congenital or acquired
previous episodes - also prev episodes of trauma, when were they
age at first event
prev surgical challenges
associated hx - FHx of bleeding disorder
hx of hereditary bleeding disorders
family members w/ similar hx
sex - determines nature of inheritance
haemophilia A and B
- inheritance
- phenotypes
- prevalence
X linked
identical phenotypes
1/10 000 (A) and 1/60 000 (B)
what determines severity of bleeding in haemophilia A and B
severity of bleeding depends on the residual coagulation factor activity
<1% severe, residual level of factor VIII and IX 1-5% moderate, 5-30% mild
clinical features of haemophila
haemarthrosis
muscle haematoma
CNS bleeding
retroperitoneal bleeding
post-surgical bleeding
clinical complications of haemophilia
synovitis
chronic haemophilic arthropathy
neurovascular compression (compartment syndromes)
other sequelae of bleeding (haemorhaggic stroke)
diagnosis of haemophilia
clinical hx
coagulation tests: APTT (activated partial thromboplastin time), PT
- prolonged APTT (coagulation factor deficiencies - reduced factor VIII or IX)
normal PT
reduced factor VIII or IX
genetic analysis - specific abnormality within the family causing the condition, used for forward planning
haemophilia treatment of bleeding diathesis
coagulation factor replacement - VIII/IX
- now almost entirely recombinant products
DDAVP - desmopression (can allow you to avoid recombinant/plasma products)
TXA - reduces the rate of clot removal
emicizumab - monoclonal ab which binds to F X and IX - provides the function of F VIII
emphasis on prophylaxis in severe haemophilia
gene therapy - produce normal F VIII/IX for the pt
treatments for symptoms of haemophilia
splints physiotherapy analgesia synovectomy joint replacement
- management of haemophilic arthropathy and muscular haematoma
complications of haemophilia treatment
viral infection from transfusion of blood products - IIIV, HBV, HCV, others - vCJD
inhibitors - develop anti F VIII ab (25% of pts), rare in F IX
DDAVP - MI, TIA, hyponatraemia (babies)
von willebrand disease
- prevalence
- severity
- inheritance
1/200
variable severity
autosomal
platelet type bleeding (mucosal)
quantitative and qualitative abnormalities of vWF
vWF deficiencies in vWD
type 1 quantitative deficiency (reduced quantity but maintains all function, reduced production/increased clearance)
type 2 (A,B,M,N) qualitative deficiency determined by the site of mutation in relation to vWF function
type 3 severe (complete) deficiency
treatment of vWD
vWF concentrate or DDAVP - elevate vWF level
TXA (tranexamic acid) - for heavy menses and after surgical procedures
topical applications - e.g. dental
OCP etc - heavy menorrhagia
when can DDAVP be used in vWD
type 1 disease
can’t use for very young children and individuals w/ significant CV risk - use vWF instead
vWF for vWD
use recombinant concentrates where possible (no dependency on blood donor)
avoid plasma derived concentrates wherever you can
acquired bleeding disorders
thrombocytopenia liver failure renal failure DIC drugs: warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin etc
