M Scully1, S Cataland2, P Coppo3, J de la Rubia4, K D Friedman5, J Kremer Hovinga6, B Lämmle7, M Matsumoto8, K Pavenski9, E Sadler10, R Sarode11, H Wu12. 1. Department of Haematology, UCLH, Cardiometabolic programme-NIHR UCLH/UCL BRC, London, UK. 2. Department of Internal Medicine, Ohio State University Hospital, Columbus, OH, USA. 3. Department of Hematology, Saint-Antoine University Hospital, Paris, France. 4. Department of Hematology, University Hospital Dr Peset, Valencia, Spain. 5. Division of Benign Hematology, Medical College of Wisconsin, Milwaukee, WI, USA. 6. Department of Hematology, Bern University Hospital, Bern, Switzerland. 7. Center for Thrombosis and Hemostasis, University Medical Center, Mainz, Germany. 8. Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan. 9. Department of Laboratory medicine, St Michael's Hospital/Research Institute, Toronto, Ontario, Canada. 10. Department of Hematology, Washington University School of Medicine, St Louis, MO, USA. 11. Department of Pathology, UT Southwestern Medical Center, Dallas, TX, USA. 12. Department of Pathology, Ohio State University Hospital, Columbus, OH, USA.
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.
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: Linus A Völker; Jessica Kaufeld; Wolfgang Miesbach; Sebastian Brähler; Martin Reinhardt; Lucas Kühne; Anja Mühlfeld; Adrian Schreiber; Jens Gaedeke; Markus Tölle; Wolfram J Jabs; Fedai Özcan; Silke Markau; Matthias Girndt; Frederic Bauer; Timm H Westhoff; Helmut Felten; Martin Hausberg; Marcus Brand; Jens Gerth; Markus Bieringer; Martin Bommer; Stefan Zschiedrich; Johanna Schneider; Saban Elitok; Alexander Gawlik; Anja Gäckler; Andreas Kribben; Vedat Schwenger; Ulf Schoenermarck; Maximilian Roeder; Jörg Radermacher; Jörn Bramstedt; Anke Morgner; Regina Herbst; Ana Harth; Sebastian A Potthoff; Charis von Auer; Ralph Wendt; Hildegard Christ; Paul T Brinkkoetter; Jan Menne Journal: Blood Adv Date: 2020-07-14
Authors: Mohammad S Abdelgawwad; Wenjing Cao; Liang Zheng; Nicole K Kocher; Lance A Williams; X Long Zheng Journal: Arterioscler Thromb Vasc Biol Date: 2018-11 Impact factor: 8.311
Authors: X Long Zheng; Sara K Vesely; Spero R Cataland; Paul Coppo; Brian Geldziler; Alfonso Iorio; Masanori Matsumoto; Reem A Mustafa; Menaka Pai; Gail Rock; Lene Russell; Rawan Tarawneh; Julie Valdes; Flora Peyvandi Journal: J Thromb Haemost Date: 2020-09-11 Impact factor: 5.824