TY - JOUR
T1 - Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies
AU - the International Working Group for Thrombotic Thrombocytopenic Purpura
AU - Scully, M.
AU - Cataland, S.
AU - Coppo, P.
AU - de la Rubia, J.
AU - Friedman, K. D.
AU - Kremer Hovinga, J.
AU - Lämmle, B.
AU - Matsumoto, M.
AU - Pavenski, K.
AU - Sadler, E.
AU - Sarode, R.
AU - Wu, H.
AU - Gale, D.
AU - Fujimura, Y.
AU - McDonald, V.
AU - Peyvandi, F.
AU - Scharrer, I.
AU - Veyradier, A.
AU - Westwood, J. P.
N1 - Publisher Copyright:
© 2016 International Society on Thrombosis and Haemostasis
PY - 2017/2/1
Y1 - 2017/2/1
N2 - 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.
AB - 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.
KW - ADAMTS-13 protein, human
KW - diagnosis, differential
KW - thrombocytopenia
KW - thrombotic microangiopathy
KW - thrombotic thrombocytopenic purpura
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U2 - 10.1111/jth.13571
DO - 10.1111/jth.13571
M3 - Article
C2 - 27868334
AN - SCOPUS:85011711363
SN - 1538-7933
VL - 15
SP - 312
EP - 322
JO - Journal of Thrombosis and Haemostasis
JF - Journal of Thrombosis and Haemostasis
IS - 2
ER -