What are thrombotic microangiopathies?¶
Answer: TMAs are a diverse group of diseases that manifest (or are defined by) microangiopathic hemolytic anemia (DAT-negative hemolysis), thrombocytopenia, and end-organ damage.
- Primary causes: inherited ADAMTS13 deficiency (congenital TTP, cTTP) and acquired ADAMTS13 deficiency (immune TTP, iTTP), plus STEC-HUS.
- Secondary causes: e.g. HELLP and systemic cancer.
- Recognition of a TMA is important because prompt treatment may reverse organ injury and improve morbidity and mortality.
- The most important TMA to learn is immune TTP, because immediate PLEX is standard of care and treatment should not be delayed.