Nephrology Dialysis Transplantation, Vol 13, Issue 1 76-81, Copyright © 1998 by Oxford University Press
M Hollenbeck, B Kutkuhn, C Aul, M Leschke, R Willers and B Grabensee
Background: Left untreated, haemolytic-uraemic
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. Because of similarities in clinical
and morphological findings, both diseases are considered as one entity
referred to as HUS-TTP syndrome. Methods: From 1974 to
January 1995, 45 adult patients received treatment for HUS-TTP at our
clinic. By stepwise logistic regression analyses, we examined how known
risk factors and plasma exchange with fresh-frozen plasma (PE) influenced
mortality and end-stage renal disease. Results: Three
of 45 patients died (7%). Though we were not able to find significant
predictors of mortality, low haemoglobin levels (5.93±0.32
vs 9.10±2.16 g/dl and high leukocyte counts
on admission (15.830±3.690 vs
11.150±4.580 &mgr;l-1) appeared to
indicate an unfavourable outcome. Regarding the development of end-stage
renal disease, PE proved to be the only favourable indicator (P=0.0001). PE
was performed in 30 patients 3-10 times (9.2±4.8,
mean±SD). Of 28 surviving patients treated with PE, only four
developed end-stage renal disease, 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 end-stage renal disease. 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. Conclusion: This retrospective clinical
study confirms the therapeutic value of plasma exchange with fresh-frozen
plasma to maintain renal function in patients with HUS-TTP. Key
words: adolescence; adult; dialysis; end-stage renal disease;
haemolytic-uraemic syndrome; mortality; plasma exchange; prednisolone;
purpura, thrombotic thrombocytopenic; therapy
ORIGINAL ARTICLES
Haemolytic-uraemic syndrome and thrombotic-thrombocytopenic purpura in adults: clinical findings and prognostic factors for death and end-stage renal disease
Clinic of Internal Medicine Department of Nephrology and Rheumatology, Department of Haematology, Oncology and Clinical Immunology, Department of Cardiology, Pneumology and Angiology, and Institute of Biostatistics, Heinrich-Heine-Universitat-Dusseldorf, Moorenstrasse 5, D-40225 Dusseldorf, Germany
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