TOTAL PLASMA EXCHANGE FOR THE MANAGEMENT OF THROMBOTIC THROMBOCYTOPENIC PURPURA IN A 13- YEAR-OLD FEMALE – A CASE PRESENTATION
Abstract:
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic
microangiopathy, affecting preferentially young women in their fourth decade. Intensive care
admission is often required due to organ dysfunction development risk and for specific critical care
measures (Plasma Exchange-PEX). In this article, we will discuss our experience with PEX in the
treatment of TTP. Case report: A previously healthy 13-year-old female presented with neurological
impairment, and suspicion of stroke. The head computed tomography (CT) scan revealed absence of
acute intracranian pathology, and biological evaluation displayed severe thrombocytopenia and
haemolytic anemia. After 24 hours, the neurological symptoms were remitted and suspicion of
thrombotic thrombocytopenic purpura was raised. The presence of ADAMTS-13 antibodies and
Moschcowitz’s pentad confirmed the diagnosis. Discussions: The distinctiveness of this case lies in the
development of the disease in a 13-year-old person, though TTP usually occurs after the age of 40.
The exact cause of ADAMTS-13 low activity could not be established. The use of a high dose of
steroids and of plasma exchange is considered to be the first line therapy, with the use of monoclonal
antibodies in refractory cases, as it was in our case. Conclusions: The primary end points of our
management was to prevent organ damage and to achieve a platelet count greater than 150 000 /μL,
as well as a normal or an almost normal lactate dehydrogenase. We achieved this by using high dose
corticosteroid therapy, filtration of approximately 50 liters of plasma in 14 PEX session and by
administration of monoclonal antibodies.
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