X Long Zheng1, Sara K Vesely2, Spero R Cataland3, Paul Coppo4, Brian Geldziler5, Alfonso Iorio6,7, Masanori Matsumoto8, Reem A Mustafa9, Menaka Pai7, Gail Rock10, Lene Russell11, Rawan Tarawneh12, Julie Valdes13, Flora Peyvandi14,15. 1. Department of Pathology and Laboratory Medicine, The University of Kansas Medical Center, Kansas City, KS, USA. 2. Hudson College of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA. 3. Department of Medicine, The Ohio State University, Columbus, OH, USA. 4. Centre de Référence des Microangiopathies Thrombotiques, Service d'Hématologie, Hôpital Saint-Antoine, Assistance Publique, Hôpitaux de Paris, Sorbonne Université, Paris, France. 5. Somerset, NJ, USA. 6. Department of Health Research Methods, Research, and Impact, McMaster University, Hamilton, ON, Canada. 7. Department of Medicine, McMaster University, Hamilton, ON, Canada. 8. Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan. 9. Department of Medicine, The University of Kansas Mediccal Center, Kansas City, KS, USA. 10. University of Ottawa, Ottawa, CA, USA. 11. Department of Intensive Care, Copenhagen University Hospital, Copenhagen, Denmark. 12. Department of Neurology, The Ohio State University Wexner Medical Center, Columbus, OH, USA. 13. Morristown, NJ, USA. 14. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy. 15. Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Abstract
BACKGROUND: Despite an increase in our understandings of pathogenesis of thrombotic thrombocytopenic purpura (TTP), the approaches for initial diagnosis and management of TTP vary significantly. OBJECTIVE: The evidence-based guidelines of the International Society on Thrombosis and Haemostasis (ISTH) are intended to support patients, clinicians, and other health care professionals in their decisions about the initial diagnosis and management of acute TTP. METHODS: In June 2018, ISTH formed a multidisciplinary panel that included hematologists, an intensive care physician, nephrologist, clinical pathologist, biostatistician, and patient representatives, as well as a methodology team from McMaster University. The panel composition was designed to minimize the potential conflicts of interests. The panel used the Grading of Recommendations Assessment, Development, and Evaluation approach and the Population, Intervention, Comparison, Outcome framework to develop and grade their recommendations. Public comments were sought and incorporated in the final document. RESULTS: The panel agreed on three recommendations covering the initial diagnosis with emphasis on the importance of ADAMTS13 testing (eg, activity, anti-ADAMTS13 IgG or inhibitor) and assessment of the pretest probability of TTP by clinical assessment and/or the risk assessment models like the PLASMIC or French score. The panel noted how availability and turnaround time of ADAMTS13 test results might affect early diagnosis and management, in particular the use of caplacizumab. CONCLUSIONS: There is a lack of high-quality evidence to support strong recommendations for the initial diagnosis and management of a suspected TTP. The panel emphasized the importance of obtaining ADAMTS13 testing in a proper clinical context. Future research should focus on how to monitor and act on ADAMTS13 levels during remission.
BACKGROUND: Despite an increase in our understandings of pathogenesis of thrombotic thrombocytopenic purpura (TTP), the approaches for initial diagnosis and management of TTP vary significantly. OBJECTIVE: The evidence-based guidelines of the International Society on Thrombosis and Haemostasis (ISTH) are intended to support patients, clinicians, and other health care professionals in their decisions about the initial diagnosis and management of acute TTP. METHODS: In June 2018, ISTH formed a multidisciplinary panel that included hematologists, an intensive care physician, nephrologist, clinical pathologist, biostatistician, and patient representatives, as well as a methodology team from McMaster University. The panel composition was designed to minimize the potential conflicts of interests. The panel used the Grading of Recommendations Assessment, Development, and Evaluation approach and the Population, Intervention, Comparison, Outcome framework to develop and grade their recommendations. Public comments were sought and incorporated in the final document. RESULTS: The panel agreed on three recommendations covering the initial diagnosis with emphasis on the importance of ADAMTS13 testing (eg, activity, anti-ADAMTS13 IgG or inhibitor) and assessment of the pretest probability of TTP by clinical assessment and/or the risk assessment models like the PLASMIC or French score. The panel noted how availability and turnaround time of ADAMTS13 test results might affect early diagnosis and management, in particular the use of caplacizumab. CONCLUSIONS: There is a lack of high-quality evidence to support strong recommendations for the initial diagnosis and management of a suspected TTP. The panel emphasized the importance of obtaining ADAMTS13 testing in a proper clinical context. Future research should focus on how to monitor and act on ADAMTS13 levels during remission.
Authors: I Mancini; P Agosti; M Boscarino; B Ferrari; A Artoni; R Palla; M Spreafico; G Crovetti; E Volpato; S Rossini; C Novelli; S Gattillo; L Barcella; M Salmoiraghi; A Falanga; F Peyvandi Journal: Orphanet J Rare Dis Date: 2022-06-23 Impact factor: 4.303
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