Haemolytic-uraemic syndrome and thrombotic-thrombocytopenic purpura in adults: clinical findings and prognostic factors for death and end-stage renal disease. Hollenbeck M, Kutkuhn B, Aul C, Leschke M, Willers R, Grabensee B. Nephrology Dialysis Transplantation 1998;13(1):76-81.

ABSTRACT:

Left untreated, hemolytic-uremic syndrome (HUS) and thrombotic-thrombocytopenic purpura (TTP) in adults have a poor prognosis with mortality rates reaching 90%. Patients who survive often develop end-stage renal disease (ESRD). Case reports and more recent prospective studies indicate that the prognosis is favorably influenced by therapy with plasma exchange (PE). Because of similarities in clinical and morphological findings, both diseases are increasingly considered as one entity referred to as HUS-TTP syndrome. This German study examined how known risk factors and PE with fresh-frozen plasma (FFP) influenced mortality and ESRD in 45 adult patients who received treatment for HUS-TTP from 1974 to 1995. Nine (20%) of the cases had prodromal gastrointestinal symptoms and the remainder had vasculitis, urinary tract infections, renal transplantation, or reproductive-related conditions. Three (7%) of 45 patients died. Although no significant predictors of mortality were identified, low hemoglobin levels and high leukocyte counts on admission appeared to indicate an unfavorable outcome. PE proved to be the only predictor of the development ESRD. PE was performed in 30 patients. Of 28 surviving patients treated with PE, only four developed ESRD, whereas dialysis was necessary in 11 of 14 patients not treated with PE. Application of PE led to an 81.8% reduction of the relative risk of developing ESRD. An additional prognostic influence of other potential risk factors such as age, sex, platelet count on admission, lactate dehydrogenase serum levels, serum creatinine, blood pressure, prodromal disease, and renal histology was not found. This retrospective clinical study confirms the therapeutic value of PE with FFP to maintain renal function in patients with HUS-TTP.