ArticleJournal of thrombosis and haemostasis : JTH2020
ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura.
Article in Journal of thrombosis and haemostasis : JTH, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 172 papers, 9 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
172 citing papers in PubMed, 9 syntheses or guidelines pooled it.
- Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach From the Writing Committee of the American Society for Apheresis: The Tenth Special Issue.Journal of clinical apheresis · 2026Guideline
- Diagnosis and monitoring of thrombotic thrombocytopenic purpura: Guidance for laboratories from the British Society for Haematology.British journal of haematology · 2026Guideline
- Advances in rapid ADAMTS13 testing for thrombotic thrombocytopenic purpura: a systematic review of diagnostic assays and the role of clinical probability tools.Frontiers in medicine · 2026Pooled it
- Complement-amplified thrombotic microangiopathy in systemic lupus erythematosus and lupus nephritis: a systematic review of 78 reported patients.Frontiers in immunology · 2026Pooled it
- Pooled it
- Alemtuzumab and thrombotic thrombocytopenic purpura: Analysis of an international surveillance database and systematic literature review.Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis · 2025Pooled it
- Effectiveness and safety of caplacizumab in acquired thrombotic thrombocytopenic purpura: health technology assessment and classification according to the methodology established in Colombia.International journal of technology assessment in health care · 2023Pooled it
- Adding caplacizumab to standard of care in thrombotic thrombocytopenic purpura: a systematic review and meta-analysis.Blood advances · 2023Pooled it
- [Chinese guideline on the diagnosis and management of thrombotic thrombocytopenic purpura (2022)].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2022Guideline
- Long-term follow-up of patients treated with caplacizumab and safety and efficacy of repeat caplacizumab use: Post-HERCULES study.Journal of thrombosis and haemostasis : JTH · 2022Trial
- Legionella Septic Cardiomyopathy Requiring Venoarterial Extracorporeal Membrane Oxygenation Followed by Presumed Complement-Mediated Thrombotic Microangiopathy: A Case Report.Journal of clinical medicine · 2026Article
- TTP-Like Syndrome and Subsequent Non-Aneurysmal Subarachnoid Hemorrhage in HbSC Disease: A Case Report.Clinical case reports · 2026Article
- Fulminant Thromboinflammatory Syndrome Following an Influenza-like Illness in an Adolescent: Clinical Insights from a Case Report.International journal of molecular sciences · 2026Article
- Article
- Delayed Recognition of Thrombotic Thrombocytopenic Purpura Following Platelet Transfusion: A Clinical Pitfall.Clinical case reports · 2026Article
- A rapid ADAMTS13 activity assay using a portable readout device.Blood vessels, thrombosis & hemostasis · 2026Article
- Immune Thrombotic Thrombocytopenic Purpura Presenting as an Acute Stroke Mimic in a Young Pakistani Man.Cureus · 2026Article
- Human monoclonal and polyclonal anti-ADAMTS13 antibodies in immune thrombotic thrombocytopenic purpura: variable mechanisms of inhibition.Journal of thrombosis and haemostasis : JTH · 2026Article
- Korean expert consensus on the management of thrombotic thrombocytopenic purpura.The Korean journal of internal medicine · 2026Article
- Analysis of causes and outcomes of principal secondary thrombotic microangiopathy: a 9-year cohort study from a tertiary pediatric center in China.Research and practice in thrombosis and haemostasis · 2026Article
112 more citing papers are in PubMed but not listed here.
Corrections and comments
- Erratum issuedErratum.2021
Authors and funding
14 authors.
Funding
Abstract
backgroundDespite an increase in our understandings of pathogenesis of thrombotic thrombocytopenic purpura (TTP), the approaches for initial diagnosis and management of TTP vary significantly.
objectiveThe 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.
methodsIn 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.
resultsThe 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.
conclusionsThere 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.
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