TTP and Microangiopathic Anaemias (3)

Question 1

What occurs here?

Which types of vessels are affected?

Why do these clots occur?

What is the effect of these clots forming in the small vessels?

How is it managed?

Answer 1

➊ Tiny clots develop throughout the body, using up platelets and causing Thrombocytopenia, Bleeding under the skin, and other systemic issues

➋ The small vessels, which is why it’s called a Microangiopathy

➌ There’s a problem with the protein ADAMTS13, which usually inactivates vWF and reduces platelet adhesion to vessel walls → vWF overactivity, and more clot formation

➍ They break up RBCs → Haemolytic Anaemia

➎ PLEX, Steroids, Rituximab

Question 2

Microangiopathic Haemolytic Anaemia (MAHA):
What occurs here?

What is usually associated with?

Answer 2

➊ Type of Intravascular haemolysis - Structural abnormalities of small blood vessels, causing mechanical destruction of RBCs as they travel through them, forming Schistocytes (seen on blood film)

➋ A thrombotic microangiopathy syndrome (e.g. TTP, Haemolytic Uraemic Syndrome), or DIC