title: 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.
  
  
  
  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.affiliation: Department of Haematology, UCLH, Cardiometabolic programme-NIHR UCLH/UCL BRC, London, UK.
authors.forename: M
authors.initials: M
authors.lastname: Scully
authors.affiliation: Department of Internal Medicine, Ohio State University Hospital, Columbus, OH, USA.
authors.forename: S
authors.initials: S
authors.lastname: Cataland
authors.affiliation: Department of Hematology, Saint-Antoine University Hospital, Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Coppo
authors.affiliation: Department of Hematology, University Hospital Dr Peset, Valencia, Spain.
authors.forename: J
authors.initials: J
authors.lastname: de la Rubia
authors.affiliation: Division of Benign Hematology, Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: K D
authors.initials: KD
authors.lastname: Friedman
authors.affiliation: Department of Hematology, Bern University Hospital, Bern, Switzerland.
authors.forename: J
authors.initials: J
authors.lastname: Kremer Hovinga
authors.affiliation: Center for Thrombosis and Hemostasis, University Medical Center, Mainz, Germany.
authors.forename: B
authors.initials: B
authors.lastname: Lämmle
authors.affiliation: Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Matsumoto
authors.affiliation: Department of Laboratory medicine, St Michael's Hospital/Research Institute, Toronto, Ontario, Canada.
authors.forename: K
authors.initials: K
authors.lastname: Pavenski
authors.affiliation: Department of Hematology, Washington University School of Medicine, St Louis, MO, USA.
authors.forename: E
authors.initials: E
authors.lastname: Sadler
authors.affiliation: Department of Pathology, UT Southwestern Medical Center, Dallas, TX, USA.
authors.forename: R
authors.initials: R
authors.lastname: Sarode
authors.affiliation: Department of Pathology, Ohio State University Hospital, Columbus, OH, USA.
authors.forename: H
authors.initials: H
authors.lastname: Wu
mesh_terms: D000071120:ADAMTS13 Protein / Q000097:blood; D000328:Adult; D001792:Blood Platelets / Q000378:metabolism; D002648:Child; D003165:Complement System Proteins; D003937:Diagnosis, Differential; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D005337:Fibrin / Q000737:chemistry; D006405:Hematology / Q000592:standards*; D006461:Hemolysis; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis; D006801:Humans; D007249:Inflammation; D010974:Platelet Aggregation; D010976:Platelet Count; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis*; D012008:Recurrence; D012074:Remission Induction; D012955:Societies, Medical; D009626:Terminology as Topic; D057049:Thrombotic Microangiopathies / Q000175:diagnosis*; D016896:Treatment Outcome; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D014841:von Willebrand Factor; D005337:Fibrin; D003165:Complement System Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS-13 protein, human; diagnosis, differential; thrombocytopenia; thrombotic microangiopathy; thrombotic thrombocytopenic purpura