Clinical Scoring Systems in Thrombotic Microangiopathies

Academic Article

Abstract

  • © 2017 by Thieme Medical Publishers, Inc. Thrombotic microangiopathies (TMAs) are a group of rare but potentially lethal hematologic disorders characterized by platelet-rich thrombi in the microvasculature. In evaluating patients with TMA, it is crucial to identify those who have thrombotic thrombocytopenic purpura (TTP), a subtype of TMA that occurs due to a severe deficiency in ADAMTS13 (a d isintegrin a nd m etalloprotease with t hrombo s pondin type 1 motif, member 13). Individuals with TTP require urgent therapeutic plasma exchange, which is associated with a significant reduction in mortality; however, at most centers, the results of ADAMTS13 activity testing are usually not available for 3 to 5 days. Given the possible need for urgent intervention and the lack of timely test results to guide therapy, the assessment of patients with TMA represents an ideal situation for the deployment of diagnostic scoring systems to predict the presence of severe ADAMTS13 deficiency. Here, we review the literature surrounding clinical prediction tools in the diagnosis and prognostication of patients with TMA, describe the experience at our center with evaluating TMA patients, and discuss the utility of clinical scoring systems for TMA in the context of patient care.
  • Authors

    Published In

    Digital Object Identifier (doi)

    Author List

  • Bendapudi PK; Upadhyay V; Sun L; Marques MB; Makar RS
  • Start Page

  • 540
  • End Page

  • 548
  • Volume

  • 43
  • Issue

  • 5