Background: The largest number of adult cases of haemolytic uraemic syndrome (HUS)/thrombotic thrombocytopenic purpura (TTP) during an Escherichia coli O157 outbreak occurred in 1996 in central Scotland. Adults who develop HUS/TTP induced by E. coli O157 tend to be elderly and have a historical mortality rate of almost 90% when treated conservatively. Therefore the decision was made to treat adults who developed HUS/TTP during this outbreak with therapeutic plasma exchange (TPE). We report our outcome with this controversial treatment.
Methods: A case definition for HUS/TTP was developed at the beginning of the outbreak. All cases meeting this definition were considered for TPE. Information on demographics, clinical features, treatment and outcome of patients was obtained by retrospective case note review.
Findings: 22 adults developed HUS/TTP. They had a mean age of 71 years. 16 cases received TPE. Six cases had contraindications to TPE or died before the procedure could be done. Ten of the 22 (45%) adults with HUS/TTP died. Five of the 16 (31%) TPE-treated cases died, four of eight aged over 70 years compared with one of eight aged less than 70 years. Premorbid illness, neurological features, treatment with ciprofloxacin or prostacyclin, and the laboratory severity of HUS/TTP were not associated with death; the number of cases, however, was too small to allow statistical conclusion.
Interpretation: The mortality rate is high in adults who develop HUS/TTP induced by E. coli O157. TPE appears to be a promising treatment that was well tolerated in our elderly patients. A national register of adult cases of HUS/TTP induced by E. coli O157 should be established.