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E.M. Staley et al.
the pathogenesis of iTTP, although the causative role of HNP1-3, histone, and complement activation in iTTP are yet to be determined. Other clinical and laboratory factors such as the elevated troponin levels and reduced Glasgow Coma Score (GCS) confer a six-fold and nine-fold increase, respectively, in the mortality of patients with iTTP, reported from the UK TTP registry.13 Our univariate analysis also demonstrated the association between an increased troponin level and mortality.
In summary, our study further demonstrates the utility of several clinical and laboratory markers including aPTT, fibrinogen, troponin, the rate of platelet and LDH normal- ization, and total protein/albumin etc. for predicting out- come in patients with iTTP. Moreover, we identified sev- eral novel biomarkers related to inflammation (e.g., VWF, HNP1-3, and histone/DNA complexes) and innate immu- nity (e.g., Bb and sC5b-9) that may be used to assess dis-
ease severity and predict long-term outcome in patients with iTTP. Our findings may help stratify patients who may benefit from more intensive care and management including twice daily TPE, early rituximab, and eclulizum- ab etc. to reduce in-hospital mortality and long-term com- plications.
Funding
This work was partially supported by grants from the National Heart, Lung, and Blood Institute (HL-26724 and HL-115187) and the Answering T.T.P. Foundation, Canada.
Acknowledgments
The collection and storage of patient samples could not have occurred without the help of the UAB apheresis nurses, the UAB Coagulation Lab, as well as the UAB Pathology residents and Transfusion Medicine fellows.
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