Acute thrombotic thrombocytopenic purpura (TTP) is an aggressive thrombotic microangiopathy that if not treated, can have a 90% mortality rate. Clinical manifestations of this disease include profound thrombocytopenia, hemolytic anemia, and end-organ dysfunction. Neurologic symptoms can occur in 80% of patients and range from mild confusion to coma (Scully et al., Br J Haematol 142:819-826). Here, we present the clinical course of a patient diagnosed with new onset acquired TTP who presented with neurologic changes that waxed and waned during her disease course. In addition to usual clinical and laboratory markers for TTP severity and activity, we also collected and analyzed the protein S100B, an astroglial protein studied as a marker for central nervous system injury and impairment of the blood-brain barrier. Our hypothesis here is that because TTP involves endovascular damage, S100B could function as a biomarker for neurologic dysfunction and ultimately, predict disease activity. As illustrated in this case, our patient's S100B levels did appear to correlate with TTP disease activity and the trajectory of this protein seemed a better predictor of cognitive function. Furthermore, increased S100B velocity seemed to be the earliest indicator of a refractory TTP disease process requiring more intensive plasma exchange (TPE) therapy regimen. Therefore, we would suggest that S100B is a promising predictive biomarker of disease activity in guiding the intensity of TPE therapy for TTP as well as cognitive function.
Keywords: S100B; thrombotic microangiopathy; thrombotic thrombocytopenic purpura.
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