Rapid ADAMTS13 availability impacts treatment for microangiopathic hemolytic anemia and thrombocytopenia

Thrombotic thrombocytopenic purpura (TTP) can present with a spectrum of clinical manifestations. When TTP is in a patient's clinical differential diagnosis, therapeutic plasma exchange (TPE) should be initiated emergently. Enzyme activity level of A Disintegrin And Metalloproteinase with a Thrombospondin type 1 motif, member 13 (ADAMTS13) in conjunction with the evolving clinical picture can guide further therapy, including duration and frequency of TPE and choice of fluid replacement. Our experience switching reference laboratories to obtain a more rapid turnaround time of ADAMTS13 activity level resulted in significant changes in clinical management, including fewer overall TPE procedures and the occasional use of albumin for a portion of the replacement fluid in patients without severe deficiency of ADAMTS13 and a low index of clinical suspicion for TTP. J. Clin. Apheresis 31:419–422, 2016.