Immune thrombotic thrombocytopenic purpura (iTTP)

Immune thrombotic thrombocytopenic purpura (iTTP) is one such form of TMA.  Patients with iTTP will characteristically present with an anemia, severe thrombocytopenia, and schistocytes in the peripheral blood in the context of neurologic, renal and even cardiac injury.  While iTTP remains a clinical diagnosis that relies on the presence of a MAHA and thrombocytopenia without an alternative clinical explanation, the diagnosis is confirmed by the finding of severely deficient (<10%) ADAMTS13 activity.  While iTTP remains very treatable, each acute iTTP episode is associated with a 10-20% risk of mortality, as well as additional acute and chronic complications of the disease.