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

Autor: 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
Rok vydání: 2017
Předmět:
Erythrocytes
Platelet Aggregation
030204 cardiovascular system & hematology
0302 clinical medicine
Pregnancy
Recurrence
hemic and lymphatic diseases
Child
Societies
Medical
Disseminated intravascular coagulation
biology
Remission Induction
Microangiopathic hemolytic anemia
Hematology
3. Good health
Treatment Outcome
ADAMTS-13 protein
human
diagnosis
differential
thrombocytopenia
thrombotic microangiopathy
thrombotic thrombocytopenic purpura
Female
Adult
Blood Platelets
medicine.medical_specialty
Thrombotic microangiopathy
Consensus
Thrombotic thrombocytopenic purpura
ADAMTS13 Protein
Hemolysis
Diagnosis
Differential
03 medical and health sciences
Von Willebrand factor
Terminology as Topic
von Willebrand Factor
medicine
Humans
Intensive care medicine
Inflammation
Fibrin
Purpura
Thrombotic Thrombocytopenic
business.industry
Platelet Count
Thrombotic Microangiopathies
Complement System Proteins
medicine.disease
Schistocyte
Transplantation
Immunology
Hemolytic-Uremic Syndrome
biology.protein
Caplacizumab
business
030215 immunology
Zdroj: JOURNAL OF THROMBOSIS AND HAEMOSTASIS
r-FISABIO: Repositorio Institucional de Producción Científica
Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
Journal of thrombosis and haemostasis : JTH
r-FISABIO. Repositorio Institucional de Producción Científica
instname
ISSN: 1538-7933
Popis: 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. SummaryBackground 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.
Databáze: OpenAIRE
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