ABSTRACT
This chapter presents a case study of a 35-year-old woman who is presented to the Emergency Department with a 3-day history of feeling generally unwell and weak. The patient presents with relatively non-specific symptoms, including fever, and has evidence of thrombocytopaenia, haemolytic anaemia and renal failure, the constellation of which should alert clinicians to a diagnosis of thrombotic thrombocytopaenic purpura (TTP). TTP is a thrombotic microangiopathy caused by deficiency of the ADAMTS13 protease, an enzyme that cleaves von Willebrand factor (VWF) multimers from endothelial cells. The deficiency of this enzyme leads to development of platelet thrombi in small vessels, producing haemolytic anaemia and thrombocytopaenia. Response to treatment is assessed by monitoring the platelet count, with normalisation expected after a week of therapy, at which point plasma exchange (PLEX) may be discontinued; glucocorticoids are typically tapered off more slowly over a period of 2-3 weeks.
