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DR. ROCK:
TTP has classically been
categorized as having a pentad presentation with
thrombocytopenia or a low platelet count, anemia,
fever, neurological signs and renal abnormalities.
Now, in the so-called adult form
of TTP the emphasis is largely on the
neurological signs. Nonetheless, the platelet microthrombi
in fulminant disease can be
widespread. And it is by no means unusual to
see renal abnormalities and, in fact, in our
first series of a hundred patients that we
randomized, as you will see in a few minutes, an
additional 35 patients or 25 percent of those
patients, and these are in the adult group of age above 18,
were anuric or ologuric at the
time of presentation and thereby would be
considered and were considered to have Hemolytic
Uremic Syndrome.
This is what the textbooks used
to say about TTP and this is what you do see,
even today, when the disease has run its course
and is truly well developed. Fortunately, we're
detecting this disorder earlier and earlier,
possibly because the awareness which has come
about through the number of clinical trials we
have developed, and also, I think, because
physicians have a true appreciation of the fact
that one can intervene, particularly if you do
so earlier in the course of the disease, with
very good outcome.
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