TTP and Microangiopathic Anaemias

Here, tiny clots develop throughout the body, using up platelets and causing thrombocytopenia, bleeding under the skin, and other systemic issues. It typically affects the small vessels, which is why it’s called a Microangiopathy. These clots occur when there's a problem with the protein, ADAMTS13, which usually inactivates vWF and reduces platelet adhesion to vessel walls. This in turn causes vWF overactivity, and more clot formation.

The clots forming in the small vessels cause them to break up the RBCs, leading to Haemolytic Anaemia.

Managed with PLEX, Steroids, Rituximab.

Microangiopathic Haemolytic Anaemia (MAHA)

This is a type of intravascular haemolysis. The structural abnormalities of small blood vessels cause mechanical destruction of RBCs as they travel through them, forming Schistocytes (seen on blood film).

It's usually associated with a thrombotic microangiopathy syndrome (e.g. TTP, Haemolytic Uraemic Syndrome), or DIC.