Case Presentation:

A 53‐year‐old man presented with fever, altered mental status, headache, and diarrhea. He had a creatinine of 3.86 mg/dL (with no known prior renal disease) and a platelet count of 8000/μL. His hemoglobin was 14.6 g/dL, haptoglobin 144 mg/dL, and lactate dehy‐drogenase 417 IU/L, with a negative peripheral blood smear. His thrombocytopenia and other findings were attributed to probable infection and dehydration. Because of the lack of schistocytes on his peripheral blood smear, thrombotic thrombocytopenic purpura (TTP) was believed less likely to be the diagnosis. After 2 days of broad‐spectrum antimicrobial therapy and intravenous fluid, his confusion worsened, and his heart rate increased to 140 bpm. His hemoglobin acutely dropped to 11.9 g/dL, his creati‐nine increased to 6.86 mg/dL and his platelets were stable at 10,000/μL. A repeat smear at this time demonstrated moderate schistocytes. The patient's diagnosis was now believed to be consistent with TTP, the patient was transferred to the intensive care unit, and both plasmapheresis and dialysis were initiated. The patient received 4 days of plasmapheresis, with improvement of hemoglobin, platelet count, and mental status. He was transferred to the floor with plans to monitor his clinical status off plasmapheresis. Two days later, the patient developed severe chest pain, and an EKG demonstrated an acute ST‐elevation myocardial infarction. While being transported to the catheterization lab, he developed a pulseless electrical activity (PEA) cardiac arrest and subsequently died.


TTP is a disease that, although rare, requires a high index of suspicion and early treatment to avoid associated morbidity and mortality. In some cases, it is a diagnosis made at autopsy with no prior clinical suspicion before death. The classic TTP pentad is microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neurologic abnormalities, renal dysfunction, and fever. However, the combination of MAHA and thrombocytopenia is considered sufficient to initiate treatment. In our case, there was no significant evidence of MAHA despite marked thrombocytopenia and the other clinical features of TTP; evidence of MAHA developed 2 days after presentation. Treatment was delayed over those 2 days, and the patient ultimately died of an associated complication. Our case supports that early treatment of possible TTP should be considered in cases without clear evidence of MAHA.


In a patient with several clinical features of TTP but no evidence of MAHA, early plasmapheresis should still be considered to reduce the likelihood of associated morbidity and mortality.


M. Craig ‐ none