Plasma Exchange for Patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) in Pusan National University Hospital (2003~2011) / 대한수혈학회지

ABSTRACT

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are related diseases with high rates of fatality. Plasma exchange therapy improves survival rates in patients with TTP and HUS. The aim of this study is to evaluate our experience in conduct of plasma exchange procedures for treatment of patients with TTP-HUS over the past nine years, and to identify risk factors for poor response to this treatment. METHODS: Between January 2003 and August 2011, 230 plasma exchange procedures were performed for treatment of 22 TTP-HUS patients at Pusan National University Hospital. We conducted a retrospective analysis of data from clinical records and plasma exchange records for these patients. RESULTS: Fourteen female patients and eight male patients were included in the study. The majority of patients (86%) had neurologic symptoms; and 41% of patients had a fever. Eight patients presented with an additional disorder; three patients presented with Systemic Lupus Erythematosus. The mean number of plasma exchange procedures was 10.5 per patient. The overall rate of mortality following plasma exchange therapy was 27% and relapse was observed in only one patient. Rate of mortality varied with different comorbid diseases. Female patients and patients who underwent fewer plasma exchange procedures tended to be unresponsive to plasma exchange therapy, but the results are not statistically significant. Aggressive treatment involving two plasma exchange procedures within 24 hours of diagnosis and choice of any replacement fluid did not show an association with improved mortality. CONCLUSION: No association of the factors analyzed with mortality rate and responsiveness to plasma exchange was observed.