The pathogenesis of Thrombotic Thrombocytopenic Purpura (TTP) is likely to be multifactorial. Management of this condition includes plasma exchange using either FFP or cryo-depleted plasma as replacement fluids.
Both are effective, however, at the present time there is insufficient data to show whether FFP or cryo-depleted plasma is more efficacious as first line therapy. Clinical trials are currently in progress.
Large daily doses are needed, usually in the order of 3L/day (one plasma volume in an adult patient). All forms of plasma contain the metalloproteinase enzyme ADAMTS13, which may be deficient or inhibited in TTP, and contributes to degrading the ultra-large multimers of VWF that cause the excessive platelet activation and consumption. Platelet transfusions are generally contraindicated.