Hemolytic uremic syndrome is characterized by the simultaneous occurre
nce of hemolytic anemia, thrombocytopenia, and renal failure. Clinical
/pathologic data, along with the treatment and outcome of 8 adult pati
ents with HUS, are described. There were 7 females and 1 male, age 30.
7 +/- 12 years; 7 were White and 1 Black. Three patients were kidney g
raft recipients, 2 of whom were receiving cyclosporine; 2 patients wer
e postpartum; 1 case followed an abortion; 1 occurred with prodromic i
nfection; and 1 case was without a causal factor All patients presente
d with hematuria and 6 with oligoanuria. Laboratory data showed hemoly
tic anemia with schistocytes, LDH values were 2584 +/- 2191 U/L, plate
lets were 79,000 +/- 40,000/mL, creatinine concentrations were 5.9 +/-
2.5 mg/dL. Renal biopsy showed thrombotic microangiopathy. Two had pr
edominant glomerular involvement, 2 showed renal cortical necrosis, 4
were marked by predominant arteriolar involvement. In 5 patients dialy
tic therapy was performed. All were treated with fresh-frozen plasma i
nfusion and 6 with plasmapheresis. Three patients died, 2 without reco
very of renal function. In conclusion, the trigger events were related
to renal transplant in 3, 2 of them taking cyclosporine; 3 with pregn
ancy; 1 to precedent infection; and 1 with no causal factor. There was
no correlation between histological form and outcome in this group of
patients. The benefit of plasmapheresis was evident in the recovery o
f the extrarenal manifestations, although tt did not change the renal
outcome. The prognosis is poor, with a high mortality (37.5%) and/or e
nd-stage renal failure (37.5%). Complete recovery of renal function wa
s obtained in 25%.