emDocs - June 22, 2015 - By Brit Long / Edited by Koyfman A and Alerhand S
"Summary
- TTP is a clinical diagnosis: suspect TTP in a sick-appearing patient with MAHA and thrombocytopenia.
- TTP is due to deficiency/inactivity of ADAMTS13.
- The classic pentad is rare, but you can depend on MAHA and low platelets for diagnosis.
- A neurologic finding, low platelets with purpura, microangiopathic hemolytic anemia (MAHA), fever, and acute renal failure/injury
- Schistocytes are present in 100% of cases at some point in the disease course.
- Treatment is plasma exchange. FFP can assist if exchange is delayed.
- Avoid platelet transfusion if possible."
http://www.emdocs.net/thrombotic-thrombocytopenic-purpura-pearls-and-pitfalls/