Deficiency of metalloproteinase, responsible for breaking down vWF multimers
Neurological symptoms
Fever
Renal failure: abnormal urinalyis, oliguria, ARF
CBC and
peripheral smear
vWF is secreted by endothelial cells in a large polymer and is rapidly cleaved by ADAMTS-13.
Plasmapheresis and steroids are the treatments of choice. Plasma infusion should be given if plasmapheresis is not immediately available.
Do NOT give platelets, as this can increase rates of intravascular thrombosis.
TTP rates of mortality are 90% if untreated.