Steven S. Dalati, MD

Disclosures

February 28, 2011

Discussion

Plasmapheresis with fresh frozen plasma is the treatment of choice for patients with TTP.[1] Before this treatment option became available, the mortality rate from TTP was more than 90%.[2] With plasmapheresis, 60%-90% of patients survive their initial episode.[2]

TTP is caused by deficient activity of the plasma metalloproteinase ADAMTS13, which is required to process von Willebrand factor. Abnormally large von Willebrand multimers accumulate along the arteriocapillary junction, causing physical damage to passing platelets and red blood cells. This condition is more common in women, and can occur at any age. Pregnancy is a known risk factor. The classic pentad of symptoms includes: (1) microangiopathic hemolytic anemia, (2) thrombocytopenia, (3) neurologic findings, (4) fever, and (5) decreased renal function.

Elevated lactate dehydrogenase (LDH) reflects hemolytic anemia, and blood smears usually reveal schistocytes. This patient presented with 4 of 5 classic findings. Although HUS may present similarly, it usually involves pronounced renal dysfunction and mild neurologic symptoms. HUS is caused by abnormal complement regulation.[2]

Patients with TTP can relapse quickly, and close monitoring by specialists is necessary. Patients also may suffer long-term consequences, such as cognitive deficits and sustained renal dysfunction.[1]

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