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

Abstract

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.

• Authors: M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu, International Working Group for Thrombotic Thrombocytopenic Purpura
• Journal: Journal of thrombosis and haemostasis : JTH (J Thromb Haemost) Vol. 15 Issue 2 Pg. 312-322 (02 2017) ISSN: 1538-7836 [Electronic] England
• PMID: 27868334 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2016 International Society on Thrombosis and Haemostasis.

Chemical References

• von Willebrand Factor
• Fibrin
• Complement System Proteins
• ADAMTS13 Protein
• ADAMTS13 protein, human

Topics

• ADAMTS13 Protein (blood)
• Adult
• Blood Platelets (metabolism)
• Child
• Complement System Proteins
• Consensus
• Diagnosis, Differential
• Erythrocytes (metabolism)
• Female
• Fibrin (chemistry)
• Hematology (standards)
• Hemolysis
• Hemolytic-Uremic Syndrome (diagnosis)
• Humans
• Inflammation
• Platelet Aggregation
• Platelet Count
• Pregnancy
• Purpura, Thrombotic Thrombocytopenic (diagnosis)
• Recurrence
• Remission Induction
• Societies, Medical
• Terminology as Topic
• Thrombotic Microangiopathies (diagnosis)
• Treatment Outcome
• von Willebrand Factor (metabolism)