Causes of Death in Patients with Thrombotic Thrombocytopenic Purpura (TTP): A Single Institution 22-Year Autopsy Cohort
Aaron N Berg, Marian A Rollins-Raval, Kendra Parker-Pitts, Larry Nichols, Jay S Raval. University of Pittsburgh Medical Center, Pittsburgh, PA
Background: TTP is a rare disease with a mortality rate that has gone from >90% to <20%, largely due to timely diagnosis and rapid initiation of plasmapheresis. As treatment successes in TTP patients have increased, treating disease relapses have also increased. While many successfully-treated patients with TTP die from other causes, others eventually succumb to this fatal disease. As post-mortem analysis of patients who die from or with a history of TTP are rare, we sought to characterize the clinical history, histopathologic findings, and causes of death in autopsies of patients with TTP performed over the past 22 years at our institution.
Design: A retrospective review of autopsy cases performed between 1/1/1990 and 4/30/2012 identified 19 patients with TTP in the cause of death or final diagnosis of autopsy reports. Medical records, autopsy reports, and histopathologic data were reviewed.
Results: 8 patients died directly as a result of their TTP; 11 patients died either as an indirect complication of or with a history of TTP. For all patients, the mean most abnormal laboratory values prior to death were as follows: platelets 27,000/µl (±28,000); hemoglobin 6.8 g/dl (±1.7); LDH 2802 U/L (±2138); creatinine 3.8 mg/dl (±2.0). ADAMTS13 activity testing results were available for 3 patients; activity was abnormally low (<67%) in all 3 cases and severely deficient (<10%) in 2. The mean hospital duration between admission and death was 22.5 days (±32.2). Plasmapheresis was utilized in 12 patients, with a range of 1-56 procedures performed over the hospital course prior to death. Histopathologic findings are reported in the table.
|Thrombi/Emboli||9||12||3||7||7 (adrenals, bowel, great vessels, liver, ovary, pancreas, uterus)|
|Hemorrhages||7||8||2||7||14 (adrenals, skin, bowel/esophageal/gastric/laryngeal mucosa, pancreas, spleen, urinary bladder submucosa)|
|Infarcts||5||4||6||3||3 (bowel, spleen)|