ArticleHaematologica2019
Clinical factors and biomarkers predict outcome in patients with immune-mediated thrombotic thrombocytopenic purpura.
Article in Haematologica, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 49 papers.
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Who cites it
49 citing papers in PubMed, 77 citations in OpenAlex.
- Von Willebrand factor and factor VIII as potential biomarkers for diagnosis and disease monitoring in chronic graft-versus-host disease.Bone marrow transplantation · 2026Trial
- Efficacy and safety of open-label caplacizumab in patients with exacerbations of acquired thrombotic thrombocytopenic purpura in the HERCULES study.Journal of thrombosis and haemostasis : JTH · 2020Trial
- NETs contribute to acute episodes but not ADAMTS13 relapses in immune-mediated thrombotic thrombocytopenic purpura.Blood vessels, thrombosis & hemostasis · 2026Article
- Rat models of thrombotic thrombocytopenic purpura reveal crucial role of placental ADAMTS13 in perinatal survival.Blood advances · 2026Article
- The complement factor B inhibitor iptacopan for relapsed and refractory immune thrombotic thrombocytopenic purpura.Thrombosis journal · 2026Article
- The landscape of ADAMTS13-related tests in thrombotic thrombocytopenic purpura: a review.Frontiers in medicine · 2026Review
- Establishing prognostic markers associated with neutrophil extracellular traps and the activated contact system in thrombotic microangiopathy.Annals of hematology · 2025Article
- Clinical Manifestations, Treatment Characteristics, and Clinical Outcomes in Patients with Immune Thrombotic Thrombocytopenic Purpura (iTTP) in a Real-World Setting: An Interim Analysis of the Turkish iTTP RegistryTurkish journal of haematology : official journal of Turkish Society of Haematology · 2025Observational
- Thrombocytopenia in Sepsis.Life (Basel, Switzerland) · 2025Review
- Genetic variants contribute to modulation of renal function in patients with immune thrombotic thrombocytopenic purpura.Blood vessels, thrombosis & hemostasis · 2024Article
- Outcomes of thrombotic thrombocytopenic purpura patients submitted to therapeutic plasma exchange in a single center in Brazil.Hematology, transfusion and cell therapy · 2024Article
- Targeting neutrophil extracellular trap accumulation under flow in patients with immune-mediated thrombotic thrombocytopenic purpura.Blood advances · 2024Article
- Mechanism underlying severe deficiency of plasma ADAMTS-13 activity in immune thrombotic thrombocytopenic purpura.Journal of thrombosis and haemostasis : JTH · 2024Article
- Pathological Mechanisms and Novel Testing Methods in Thrombotic Thrombocytopenic Purpura.Biomedicines · 2024Review
- Stress-induced hyperglycemia is associated with the mortality of thrombotic thrombocytopenic purpura patients.Diabetology & metabolic syndrome · 2024Article
- A Systematic Review of the Epidemiology and Disease Burden of Congenital and Immune-Mediated Thrombotic Thrombocytopenic Purpura.Journal of blood medicine · 2024Review
- Coagulation factor VIII regulates von Willebrand factor homeostasis invivo.Journal of thrombosis and haemostasis : JTH · 2023Article
- A Long-Term Follow-Up Study in Immune-Mediated Thrombotic Thrombocytopenic Purpura: What Are the Outcomes?Journal of clinical medicine · 2023Article
- Animal models for thrombotic thrombocytopenic purpura: a narrative review.Annals of blood · 2023Article
- Immune thrombotic thrombocytopenic purpura: pathogenesis and novel therapies: a narrative review.Annals of blood · 2023Article
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
Abstract
Immune-mediated thrombotic thrombocytopenic purpura is characterized by severe thrombocytopenia and microangiopathic hemolytic anemia. It is primarily caused by immunoglobin G type autoantibodies against ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor. However, reliable markers predictive of patient outcomes are yet to be identified. Seventy-three unique patients with a confirmed diagnosis of immune-mediated thrombotic thrombocytopenic purpura between April 2006 and December 2017 were enrolled from the Univeristy of Alabama at Birmingham Medical Center. Clinical information, laboratory values, and a panel of special biomarkers were collected and/or determined. The results demonstrated that the biomarkers associated with endothelial injury (e.g., von Willebrand factor antigen and collagen-binding activity), acute inflammation (e.g., human neutrophil peptides 1-3 and histone/deoxyribonucleic acid complexes), and activation of the complement alternative pathway (e.g., factors Bb and iC3b) were all significantly increased in patients with acute immune-mediated thrombotic thrombocytopenic purpura compared to those in the healthy controls. Moreover, failure to normalize platelet counts within 7 days or failure to markedly reduce serum lactate dehydrogenase by day 5, low total serum protein or albumin, and high serum troponin levels were also predictive of mortality, as were the prolonged activated partial thromboplastin time, high fibrinogen, and elevated serum lactate dehydrogenase, Bb, and sC5b-9 on admission. These results may help to stratify patients for more intensive management. The findings may also provide a framework for future multicenter studies to identify valuable prognostic markers for immune-mediated thrombotic thrombocytopenic purpura.
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