Thrombosis & Bleeding Disorders (haemophilia + VWF Disease) Flashcards
What are the 2 types of thrombosis?
Arterial and venous
Arterial
What causes the blockage?
What symptoms occur as a result?
Atherogenesis inside arteries
Ischemia to organs/tissues
cyanotic + cold
Venous
What causes the blockage?
What symptoms occur as a result?
virchows triad
Occluded venous drainage
red + warm
What are the 3 causes & consequences of Arterial blockage?
Cardiovascular - MI (+ angina), IHD induces
Cerebrovascular - Ischemic stroke + TIA
Peripheral = PVD
What are the 2 causes & consequences of venous blockage?
DVT - thrombus deep in leg
PE - emboli from DVT thrombus
What is bleeding dysfunction?
Conditions when you bleed too easily
What are 3 types of bleeding dysfunction?
Over anticoagulation
DIC
Inherited conditions
Examples of over anticoagulation meds and mechanisms
Heparin - binds to antithrombin 3 & inactivates factor x (10)
Aspirin - COX inhibitor
Clopidogrel - P2Y12 inhibitor
Thrombolytics; alteplase
What is DIC and how does it cause bleeding?
Disseminated intravascular Coagulation disorder
Microvascular thrombosis consumes clotting factors + high systemic bleed risk
What is the MC inherited bleeding disorder?
VWF Disorder
Inherited conditions causing bleeding dysfunction?
Haemophilia A & B
Von Willebrand disease
What causes Haemophilia?
X linked recessive tissue factor deficiency
What are the types of haemophilia?
Which is MC?
A and B
A is MC
Mostly males (no male - male transmission)
A=f8
B=f9
Symptoms of haemophilia
Spontaneous bleeds
haemarthrosis (bleeding into joint spaces) - compartment syndrome
Very easy bruising
Epistaxis (bleeding from nose)
Presents early in childhood/neonates (eg, ICH, haemoatomas, Cord bleeding)
Diagnosis of Haemophilia
Normal PT (prothrombin time) - extrinsic and common pathway
High APTT (as only intrinsic pathway affected) - intrinsic + common
low f8 or low f9 assay
Prenatal = CVS DNA testing
Treatment of haemophilia
A = IV F8 + desmopressin (relaxes F8 stored in vessel wall)
B = IV F9
what is medications are good to avoid in haemophilia Px?
aspirin
Nsaids
IM injections
What is VWF Disease?
Autodominant mutation of VWF gene on chromosome 12
M+F
How does the pathology of VWF cause the symptoms?
and Sx?
Low VWF (responsible for basis of platelet plug) = more spontaneous bleeds + bruising
Epistaxis, bleeding gums, easy bruising, menorrhagia, bleeding after surgical procedures
Diagnosis of VWF disease
Normal PT
High APTT
Normal F8/9 assay
low VWF
Treatment of VWF disease
Non curable
IV VWF + Desmopressin (increased release of VWF from endothelial Weibelpalade bodies)
What is DIC?
What does it stand for?
Disseminated intravascular coagulation
massive activation of coagulation cascade, a crisis
Functional thrombocytopenia (VS shock, infection, NBP RASH, FEVER, BRUISING)
What is DIC due to?
Trauma, sepsis (eg. meningococcal meningitis), Malignancy
What happens in DIC to increase bleed risk?
Platelets unneccessarily consumed + microthrombase in small BVs
lack of systemic viable platelets = bleed risk
What is going to be low/high In the body?
low fibrinogen and platelets
high D dimer
High APTT + PT
Treatment for DIC
FFP (replace clotting factor)
Cryoprecipitate (replace fibrinogen)
Platelet transfusion
RBC transfusion if bleeding
