Total Plasma Exchange for the Management of Thrombotic Thrombocytopenic Purpura in a 13-Year-Old Female – A Case Presentation

Abstract

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.