HAEM - Clotting and Bleeding Disorders Flashcards
What is Haemophilia A
Factor VIII deficiency
Inheritance Haemophilias
X linked recessive
+ high rates new mutations)
What is Haemophilia B
Factor IX deficiency
Which haemophilia is more common
A
Clinical features of haemophilias (3)
Major bleeds from minor trauma
Rec haemarthroses –> arthropathies
Compartment syndrome/nn palsies
Ix Haemophilias (2)
Raised APTT
+ Low VIII/IX on assays
Mx Haemopilias - general (2)
Avoid NSAIDs
Avoid IM injections
Mx minor bleed - haemophilias (2)
Compression + elevation
Desmopressin
Mx major bleed - haemophilias
Recombinant factor 8/9 to raise factors to 50% of normal
Mx life threatening bleed - haemophilia
Recombinant factor 8/9 to raise factors to 100% of normal
What is von willebrands disease
Abscence of vWF –> platelet type disorder
PS vWD
bleeding post op
Menorrhagia
Epistaxis
Difference between AD and AR vWD
AR - complete loss vWF (20%)
AD = less severe depletion (80%)
Ix vWD (2)
APTT increase
INR/platelets within normal limit
Mx vWD
Expert Mx
Transenamic acid for minor bleed
Desmopressin/recom factor 8 if more severe)
Site of bleed + clue it gives for condition - mm/joint
Coagulation issue
Site of bleed + clue it gives for condition - purpura/epistaxis/menorrhagia/GI haemorrhage
Platelet issue/vWd
Site of bleed + clue it gives for condition - rec bleeds @ 1 site
Local endothelial abnormality
What does bleeding immediately after surgery tell you
= Platelet issue
What does bleeding several hours after surgery tell you
= coagulation issue
Clopidogrel mode of action
Irreversible modification of platelet receptor
Dipyrimadole
PDE inhibitor (affects aggregation)
Mode of action Heparin
Activates AT3 –> inactivation of thrombin + X
Desmopression mode of action
Stimulates exocytosis of vWF
Hence is useful in vWD/Haemophilia A
How long does Warfarin take to have an effect
up to 5 days
C/I Warfarin (4)
Peptic ulcer disease
Bleeding disorders
Severe HTN
Pregnancy
Target INR - Warfarin for DVT/PE
2-3
Target INR - Warfarin for AF
2-3
Target INR - Warfarin for rec DVT/PE
3-4
Target INR - Warfarin for prosthetic heart valves
3-4
NOACs - Xa inhibtors (3)
The Xabans
Apixaban
Rivaroxaban
Edoxaban
How are Xaban’s reversed
Andexanet alfa
Mode of action dabigatran
Thrombin inhibitor
Reversal of dabigatran by:
Idarucizumab
Which NOACs are approved for the 2’’ prevention in pt w/ AF
Dabigatran
Apixaban
Rivaroxaban
+ves of NOACs (4)
Rapid onset coag
> Predictable pharmacokinetics
Less interactions w/ foods/Dx
Dont need reg coag monitoring