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 hemolyticâuremic 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.
Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies / 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, D. Gale, Y. Fujimura, V. Mcdonald, F. Peyvandi, I. Scharrer, A. Veyradier, J.P. Westwood. - In: JOURNAL OF THROMBOSIS AND HAEMOSTASIS. - ISSN 1538-7933. - 15:2(2017 Feb), pp. 312-322.
|Titolo:||Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies|
|Parole Chiave:||ADAMTS-13 protein, human; diagnosis, differential; thrombocytopenia; thrombotic microangiopathy; thrombotic thrombocytopenic purpura; ADAMTS13 protein; adult; blood platelets; child; complement system proteins; consensus; diagnosis, differential; erythrocytes; female; fibrin; hematology; hemolysis; hemolytic-uremic syndrome; humans; inflammation; platelet aggregation; platelet count; pregnancy; purpura, thrombotic thrombocytopenic; recurrence; remission induction; societies, medical; terminology as topic; thrombotic microangiopathies; treatment outcome; von Willebrand factor; hematology|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||feb-2017|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1111/jth.13571|
|Appare nelle tipologie:||01 - Articolo su periodico|
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