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

Inherited conditions causing bleeding dysfunction?

Answer 10

Haemophilia A & B
Von Willebrand disease

Question 11

What causes Haemophilia?

Answer 11

X linked tissue factor deficiency

Question 12

What are the types of haemophilia?
Which is MC?

Answer 12

A and B
A is MC
Mostly males
A=f8
B=f9

Question 13

Symptoms of haemophilia

Answer 13

Spontaneous bleeds
haemarthrosis (bleeding into joint spaces)
Very easy bruising
Epistaxis (bleeding from nose)

Question 14

Diagnosis of Haemophilia

Answer 14

Normal PT (prothrombin time) - extrinsic and common pathway

High APTT (as only intrinsic pathway affected) - intrinsic + common

low f8 or low f9 assay

Question 15

Treatment of haemophilia

Answer 15

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

Question 16

What is VWF Disease?

Answer 16

Autodominant mutation of VWF gene on chromosome 12

Question 17

What is the MC inherited bleeding disorder?

Answer 17

VWF Disorder

Question 18

How does the pathology of VWF cause the symptoms?

Answer 18

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

Question 19

Diagnosis of VWF disease

Answer 19

Normal PT
High APTT
Normal F8/9 assay
low VWF

Question 20

Treatment of VWF disease

Answer 20

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

Question 21

What is DIC?
What does it stand for?

Answer 21

Disseminated intravascular coagulation
massive activation of coagulation cascade, a crisis

Question 22

What is DIC due to?

Answer 22

Trauma, sepsis (eg. meningococcal meningitis), Malignancy

Question 23

What happens in DIC to increase bleed risk?

Answer 23

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

Question 24

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

Answer 24

low fibrinogen and platelets
high D dimer

Question 25

Treatment for DIC

Answer 25

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