Background
- One of the thrombotic microangiopathies
Pathophysiology
- Caused by acquired or inherited deficiency of the ADAMTS13 metalloprotease
- Acquired is via autoantibody
- ADAMTS13 normally breaks larger von Willebrand factor multimers into smaller particles
- This deficiency results in increased high-molecular-weight vWF, which aggregates and activates platelets
Clinical Manifestations
- Presents as a triad of fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic deficits, and acute renal failure
- AKI is typically less severe than HUS
- Coagulation parameters are typically less abnormal than in DIC
- In inherited ADAMTS13 deficiency, symptoms tend to start during adolescence and present in episodes
