ReviewHematology. American Society of Hematology. Education Program2018
Clinical and laboratory diagnosis of TTP: an integrated approach.
Review in Hematology. American Society of Hematology. Education Program, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 91 papers, 3 of them syntheses that pooled it.
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Who cites it
91 citing papers in PubMed, 3 syntheses or guidelines pooled it.
- 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
- Thrombotic thrombocytopenic purpura (TTP) after COVID-19 vaccination: A systematic review of reported cases.Thrombosis research · 2022Pooled it
- [Chinese guideline on the diagnosis and management of thrombotic thrombocytopenic purpura (2022)].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2022Guideline
- Recognizing thrombotic thrombocytopenic purpura beyond the pentad.Oxford medical case reports · 2026Article
- Unintended Interruption of Caplacizumab Therapy Can Compromise Outcomes in Immune-Mediated Thrombotic Thrombocytopenic Purpura: Lessons from Two Clinical Cases.Journal of clinical medicine · 2026Article
- Revisiting clinical scoring tools for the diagnosis of thrombotic thrombocytopenic purpura (TTP).Journal of thrombosis and thrombolysis · 2026Review
- Article
- The complement factor B inhibitor iptacopan for relapsed and refractory immune thrombotic thrombocytopenic purpura.Thrombosis journal · 2026Article
- [Caplacizumab combined with therapeutic plasma exchange, glucocorticoids, and rituximab for refractory thrombotic thrombocytopenic purpura: a case report and literature review].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2026Review
- Pregnancy-Associated Thrombotic Thrombocytopenic Purpura: Diagnostic Pitfalls, Therapeutic Strategies, and Emerging Paradigms.Biomedicines · 2026Review
- Article
- Thrombotic thrombocytopenic purpura: a Trojan horse of acute leukemia?: a case report.Journal of medical case reports · 2026Article
- The landscape of ADAMTS13-related tests in thrombotic thrombocytopenic purpura: a review.Frontiers in medicine · 2026Review
- Effectiveness of caplacizumab in the first-line treatment for acquired thrombotic thrombocytopenic purpura: single center experience.Therapeutic advances in hematology · 2026Article
- Case report: Butylphthalide-associated severe thrombocytopenia, a rare adverse drug reaction.Frontiers in pharmacology · 2026Article
- Current standards and novel concepts in thrombotic thrombocytopenic purpura.Frontiers in medicine · 2026Review
- Case report: Caplacizumab without plasma exchange for severe thrombotic thrombocytopenic purpura.Frontiers in drug delivery · 2026Article
- Addition of ADAMTS13 testing to cryptogenic stroke evaluation: index case and single-center screening initial experience.Blood advances · 2025Article
- Article
- The impact of the COVID-19 pandemic on incidence and clinical presentation of thrombotic microangiopathies: data from a laboratory centralizing ADAMTS-13 testing in Quebec.Orphanet journal of rare diseases · 2025Article
31 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
2 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Willebrand cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), leading to formation of platelet-rich thrombi in the microvasculature. Prompt initiation of appropriate therapy, particularly plasma exchange, may be life-saving. Diagnosis of TTP is challenging because of its diverse clinical manifestations, overlap in clinical presentation with other thrombotic microangiopathies, and limited availability of ADAMTS13 testing. Clinical prediction scores have been developed to estimate the pretest probability of severe ADAMTS13 deficiency and may be used as an adjunct to clinical judgment to guide initial management decisions. An ADAMTS13 activity level of less than 10% supports the diagnosis of TTP in appropriate clinical contexts, but many centers do not offer testing in-house and must send out the test to a reference laboratory with a turnaround time of several days. In such instances, initial management decisions must be made without the benefit of laboratory testing. In patients with TTP, inhibitor tests may be useful for distinguishing immune-mediated from congenital TTP. In this article, we review the epidemiology, natural history, and clinical presentation of TTP and laboratory assays for TTP including ADAMTS13 activity and inhibitor assays. We also describe an evidence-based approach to the evaluation of a patient with suspected TTP that integrates clinical and laboratory assessment.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.