++
Thrombotic thrombocytopenic purpura (TTP) was previously defined by the following pentad of symptoms: (1) microangiopathic hemolytic anemia, (2) thrombocytopenia, (3) renal abnormalities, (4) fever, and (5) neurologic abnormalities. However, at initial presentation, only 10% of acute TTP patients have all of these symptoms. Most TTP patients will have severe thrombocytopenia (usually < 30,000/mm3), microangiopathic hemolytic anemia (schistocytes in the blood smear), and a constellation of clinical findings (petechia, purpura, neurologic symptoms, myocardial ischemia, mesenteric ischemia, and renal abnormalities). Approximately 50% of TTP presentations have an associated comorbidity that may trigger the disease (autoimmune diseases, antiphospholipid syndrome, pregnancy, medications, HIV infection, pancreatitis, malignancy, and organ transplantation), whereas the other 50% are idiopathic.
+++
Management and Disposition
++
The cornerstone of primary TTP therapy is total plasma exchange transfusion (plasmapheresis) with fresh frozen plasma. Emergent consultation with hematology will establish the most expeditious management strategy available.
++
Total plasma exchange transfusions have increased TTP survival rate to 80% to 90%.
Understanding the varied presentation of TTP patients will lower your threshold to identify them earlier and expedite definitive treatment.
Pediatric TTP cases represent only 10% of all TTP cases and are often misdiagnosed.
++