ArticleInternational journal of hematology2023
Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) in Japan 2023.
Article in International journal of hematology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 39 citations in OpenAlex.
- Diagnosis and monitoring of thrombotic thrombocytopenic purpura: Guidance for laboratories from the British Society for Haematology.British journal of haematology · 2026Guideline
- Postpartum Atypical Hemolytic Uremic Syndrome Complicating β-Thalassemia Intermedia: A Case Report and Literature Review.Journal of clinical medicine · 2026Article
- Severe Immune-Mediated Thrombotic Thrombocytopenic Purpura With High-Titer ADAMTS13 Inhibitors: A Case Report.Cureus · 2026Article
- Secondary thrombotic thrombocytopenic purpura following severe infection: a case report.World journal of emergency medicine · 2026Article
- Korean expert consensus on the management of thrombotic thrombocytopenic purpura.The Korean journal of internal medicine · 2026Article
- Complement Dysregulation in Kidney Diseases: Mechanisms, Biomarkers, and Emerging Targeted Therapies.International journal of molecular sciences · 2026Review
- Thrombotic thrombocytopenic purpura as a complication of pembrolizumab: a case report and literature Review.Annals of hematology · 2026Review
- Validation of plasmin-cleaved VWF as a biomarker for microthrombosis in Japanese patients with acute immune-mediated TTP.Blood vessels, thrombosis & hemostasis · 2026Article
- From dog bite to dialysis: complement-mediated haemolytic uraemic syndrome .BMJ case reports · 2026Article
- Establishment of a novel lupus mesenteric vasculitis mouse model and exploration of its exacerbation mechanisms.Frontiers in immunology · 2026Article
- Natural History and Treatment Outcomes of Congenital Thrombotic Thrombocytopenic Purpura: A Retrospective Longitudinal Cohort Study.Journal of health economics and outcomes research · 2026Article
- Thrombotic thrombocytopenic purpura and macrophage activation syndrome secondary to Sjögren's syndrome: a case report.Frontiers in immunology · 2026Article
- Article
- All-Cause and Exacerbation-Related Readmissions in Thrombotic Microangiopathy Requiring Plasmapheresis: A Population-Based Cohort StudyTurkish journal of haematology : official journal of Turkish Society of Haematology · 2025Article
- Predicting Outcomes and Optimizing Therapy in Thrombotic Thrombocytopenic Purpura: Insights on Caplacizumab Use from a Romanian Hematology Center.Journal of clinical medicine · 2025Article
- Microangiopathic Anemia.Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie · 2025Review
- Article
- Early administration of caplacizumab combined with plasma exchange for thrombotic microangiopathy due to malignant hypertension: a case report.CEN case reports · 2025Article
- Outcome of proliferative lupus nephritis with thrombotic microangiopathy; An ambispective observational single-center study.BMC nephrology · 2025Observational
- Thrombotic Thrombocytopenic Purpura in Pediatric Patients.Biomedicines · 2025Review
Corrections and comments
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Authors and funding
13 authors at 9 institutions in 1 country.
Funding
Abstract
Thrombotic thrombocytopenic purpura (TTP) can rapidly become a life-threatening condition, and the importance of its appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has become internationally accepted as a diagnostic criterion for TTP. TTP is classified as immune-mediated TTP (iTTP) if the patient is positive for anti-ADAMTS13 autoantibodies, and as congenital TTP (cTTP) if ADAMTS13 gene abnormalities are detected. Fresh frozen plasma (FFP) transfusion is performed in patients with cTTP to supplement ADAMTS13. Plasma exchange therapy using FFP is conducted in patients with iTTP to supplement ADAMTS13 and to remove both anti-ADAMTS13 autoantibodies and unusually large von Willebrand factor (VWF) multimers. To suppress autoantibody production, corticosteroid therapy is administered in conjunction with plasma exchange. The monoclonal anti-CD-20 antibody rituximab is effective in patients with iTTP. In addition, caplacizumab, an anti-VWF A1 domain nanobody, has a novel mechanism of action, involving direct inhibition of platelet glycoprotein Ib-VWF binding. The recommended first-line treatments of iTTP in Japan are plasma exchange and corticosteroids, as well as caplacizumab.
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