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Blood, 15 August 2000, Vol. 96, No. 4, pp. 1223-1229
HOW I TREAT
How I treat patients with thrombotic thrombocytopenic
purpura-hemolytic uremic syndrome
James N. George
From the Hematology-Oncology Section, The University of
Oklahoma Health Sciences Center, Oklahoma City, OK.
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic
syndrome (HUS) are, in adults, clinically and pathologically indistinguishable except for the severity of renal failure. They are
best described as a single disorder, TTP-HUS, because the diagnostic
evaluation and initial management are the same. Treatment with plasma
exchange, available for more than 20 years, has dramatically altered
the course of disease in adults with TTP-HUS. Plasma exchange has
improved survival rates from 10% to between 75% and 92%, creating urgency for the initiation of treatment. This has resulted in decreased
stringency of diagnostic criteria, which in turn has resulted in a
broader spectrum of disorders for which the diagnosis of TTP-HUS is
considered. Long-term follow-up has revealed increasing frequencies of
relapse and of chronic renal failure. Although the increased survival
rate is dramatic and recent advances in understanding the pathogenesis
of these syndromes are remarkable, clinical decisions remain empirical.
Therefore, the management decisions for patients with suspected TTP-HUS
rely on individual experience and opinion, resulting in many different
practice patterns. Multipractice clinical trials are required to define
optimal management.
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