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None of the above: thrombotic microangiopathy beyond TTP and HUS

Camila Masias, Sumithira Vasu and Spero R. Cataland

Abstract

Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%). An increased awareness of drug-induced TMA is also essential as the key to their diagnosis more often is an appropriately detailed medical history to inquire about potential exposures. Widespread inflammation and endothelial damage are central in the pathogenesis of the thrombotic microangiopathy (TMA), with the treatment directed at the underlying disease if possible. TMA presentations in the critically ill, drug-induced TMA, cancer-associated TMA, and hematopoietic transplant-associated TMA (TA-TMA) and their specific treatment where applicable will be discussed in this manuscript. A complete assessment of all the potential etiologies for the TMA findings including acquired TTP will allow for a more accurate diagnosis, and prevent prolonged or inappropriate treatment with plasma exchange therapy when it is less likely to be successful.

  • Submitted November 9, 2016.
  • Accepted February 21, 2017.