Thrombosis & Bleeding Disorders (haemophilia + VWF Disease)

Question 1

What are the 2 types of thrombosis?

Answer 1

Arterial and venous

Question 2

Arterial
What causes the blockage?
What symptoms occur as a result?

Answer 2

Atherogenesis inside arteries

Ischemia to organs/tissues
cyanotic + cold

Question 3

Venous
What causes the blockage?
What symptoms occur as a result?

Answer 3

virchows triad

Occluded venous drainage
red + warm

Question 4

What are the 3 causes & consequences of Arterial blockage?

Answer 4

Cardiovascular - MI (+ angina), IHD induces

Cerebrovascular - Ischemic stroke + TIA

Peripheral = PVD

Question 5

What are the 2 causes & consequences of venous blockage?

Answer 5

DVT - thrombus deep in leg

PE - emboli from DVT thrombus

Question 6

What is bleeding dysfunction?

Answer 6

Conditions when you bleed too easily

Question 7

What are 3 types of bleeding dysfunction?

Answer 7

Over anticoagulation
DIC
Inherited conditions

Question 8

Examples of over anticoagulation meds and mechanisms

Answer 8

Heparin - binds to antithrombin 3 & inactivates factor x (10)
Aspirin - COX inhibitor
Clopidogrel - P2Y12 inhibitor
Thrombolytics; alteplase

Question 9

What is DIC and how does it cause bleeding?

Answer 9

Disseminated intravascular Coagulation disorder

Microvascular thrombosis consumes clotting factors + high systemic bleed risk

Question 10

What is the MC inherited bleeding disorder?

Answer 10

VWF Disorder

Question 11

Inherited conditions causing bleeding dysfunction?

Answer 11

Haemophilia A & B
Von Willebrand disease

Question 12

What causes Haemophilia?

Answer 12

X linked recessive tissue factor deficiency

Question 13

What are the types of haemophilia?
Which is MC?

Answer 13

A and B
A is MC
Mostly males (no male - male transmission)
A=f8
B=f9

Question 14

Symptoms of haemophilia

Answer 14

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)

Question 15

Diagnosis of Haemophilia

Answer 15

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

Question 16

Treatment of haemophilia

Answer 16

A = IV F8 + desmopressin (relaxes F8 stored in vessel wall)
B = IV F9

Question 17

what is medications are good to avoid in haemophilia Px?

Answer 17

aspirin
Nsaids
IM injections

Question 18

What is VWF Disease?

Answer 18

Autodominant mutation of VWF gene on chromosome 12
M+F

Question 19

How does the pathology of VWF cause the symptoms?
and Sx?

Answer 19

Low VWF (responsible for basis of platelet plug) = more spontaneous bleeds + bruising

Epistaxis, bleeding gums, easy bruising, menorrhagia, bleeding after surgical procedures

Question 20

Diagnosis of VWF disease

Answer 20

Normal PT
High APTT
Normal F8/9 assay
low VWF

Question 21

Treatment of VWF disease

Answer 21

Non curable
IV VWF + Desmopressin (increased release of VWF from endothelial Weibelpalade bodies)

Question 22

What is DIC?
What does it stand for?

Answer 22

Disseminated intravascular coagulation
massive activation of coagulation cascade, a crisis
Functional thrombocytopenia (VS shock, infection, NBP RASH, FEVER, BRUISING)

Question 23

What is DIC due to?

Answer 23

Trauma, sepsis (eg. meningococcal meningitis), Malignancy

Question 24

What happens in DIC to increase bleed risk?

Answer 24

Platelets unneccessarily consumed + microthrombase in small BVs
lack of systemic viable platelets = bleed risk

Question 25

What is going to be low/high In the body?

Answer 25

low fibrinogen and platelets
high D dimer
High APTT + PT

Question 26

Treatment for DIC

Answer 26

FFP (replace clotting factor)
Cryoprecipitate (replace fibrinogen)
Platelet transfusion
RBC transfusion if bleeding