Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies

the International Working Group for Thrombotic Thrombocytopenic Purpura

Research output: Contribution to journalArticlepeer-review

335 Scopus citations


Essentials An international collaboration provides a consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given. Summary: Background Thrombotic thrombocytopenic purpura (TTP) and hemolytic–uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates. The most common cause is disseminated intravascular coagulation, which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia, including cancer, infection, transplantation, drug use, autoimmune disease, and pre-eclampsia and hemolysis, elevated liver enzymes and low platelet count syndrome in pregnancy. Despite overlapping clinical presentations, TTP and HUS have distinct pathophysiologies and treatment pathways. Objectives To present a consensus document from an International Working Group on TTP and associated thrombotic microangiopathies (TMAs). Methods The International Working Group has proposed definitions and terminology based on published information and consensus-based recommendations. Conclusion The consensus aims to aid clinical decisions, but also future studies and trials, utilizing standardized definitions. It presents a classification of the causes of TMA, and criteria for clinical response, remission and relapse of congenital and immune-mediated TTP.

Original languageEnglish (US)
Pages (from-to)312-322
Number of pages11
JournalJournal of Thrombosis and Haemostasis
Issue number2
StatePublished - Feb 1 2017


  • ADAMTS-13 protein, human
  • diagnosis, differential
  • thrombocytopenia
  • thrombotic microangiopathy
  • thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Hematology


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