Observational studyThrombosis and haemostasis2018
Relapse Rate in Survivors of Acute Autoimmune Thrombotic Thrombocytopenic Purpura Treated with or without Rituximab.
Observational study in Thrombosis and haemostasis, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
What it found
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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.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 41 citations in OpenAlex.
- Comparison of the Long-Term Remission of Rituximab and Conventional Treatment for Acquired Thrombotic Thrombocytopenic Purpura: A Systematic Review and Meta-Analysis.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisPooled it
- The efficacy and safety of caplacizumab in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura: an open-label phase 2/3 study.International journal of hematology · 2023Trial
- Therapeutic Plasma Exchange in Immune-Mediated Thrombotic Thrombocytopenic Purpura: From Cornerstone to Contextualized Therapy.Journal of clinical medicine · 2026Review
- The landscape of ADAMTS13-related tests in thrombotic thrombocytopenic purpura: a review.Frontiers in medicine · 2026Review
- Evidence for the use of anti-CD20 therapeutic antibodies in immune thrombotic thrombocytopenic purpura management: a review.Frontiers in pharmacology · 2026Review
- GC1126A, a novel ADAMTS13 mutein, evades autoantibodies in immune-mediated thrombotic thrombocytopenic purpura.Scientific reports · 2025Article
- Experience in emergency management of first-episode immune thrombotic thrombocytopenic purpura over the past 21 years: a single-center retrospective study.Frontiers in immunology · 2025Article
- A Systematic Review of the Epidemiology and Disease Burden of Congenital and Immune-Mediated Thrombotic Thrombocytopenic Purpura.Journal of blood medicine · 2024Review
- A Long-Term Follow-Up Study in Immune-Mediated Thrombotic Thrombocytopenic Purpura: What Are the Outcomes?Journal of clinical medicine · 2023Article
- Risk of relapse after SARS-CoV-2 vaccine in the Milan cohort of thrombotic thrombocytopenic purpura patients.Haematologica · 2023Article
- Immune thrombotic thrombocytopenic purpura: pathogenesis and novel therapies: a narrative review.Annals of blood · 2023Article
- ADAMTS13 and Non-ADAMTS13 Biomarkers in Immune-Mediated Thrombotic Thrombocytopenic Purpura.Journal of clinical medicine · 2023Review
- A novel von Willebrand factor multimer ratio as marker of disease activity in thrombotic thrombocytopenic purpura.Blood advances · 2023Observational
- Successful preventive treatment with cyclosporine in a patient with relapsed/refractory immune-mediated thrombotic thrombocytopenic purpura: a case report and review of the literature.International journal of hematology · 2022Review
- Comparison of the efficacy and safety of caplacizumab versus placebo in thrombotic thrombocytopenic purpura: a meta-analysis and systematic review based on randomized controlled trials.Annals of translational medicine · 2022Article
- Severe Thrombotic Thrombocytopenic Purpura (TTP) with Organ Failure in Critically Ill Patients.Journal of clinical medicine · 2022Review
- A prognostic model (BATAP) with external validation for patients with transplant-associated thrombotic microangiopathy.Blood advances · 2021Article
- Focus on Key Issues in Immune Thrombotic Thrombocytopenic Purpura: Italian Experience of Six Centers.Journal of clinical medicine · 2021Article
- Article
- Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management.Journal of clinical medicine · 2021Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAutoimmune thrombotic thrombocytopenic purpura (iTTP) is caused by autoantibody-mediated severe a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) deficiency leading to micro-angiopathic haemolytic anaemia (MAHA) and thrombocytopenia with organ damage. Patients survive with plasma exchange (PEX), fresh frozen plasma replacement and corticosteroid treatment. Anti-CD20 monoclonal antibody rituximab is increasingly used in patients resistant to conventional PEX or relapsing after an acute bout.
objectiveThis retrospective observational study focused on the relapse rate and possible influencing factors including treatment with rituximab first introduced in 2003. PATIENTS AND
methodsSeventy patients treated between January 2003 and November 2014 were evaluated. Number, duration, clinical manifestations, laboratory data and treatment of acute episodes were documented. Diagnostic criteria of acute iTTP were thrombocytopenia, MAHA, increased lactate dehydrogenase and severe ADAMTS13 deficiency.
resultsFifty-four female and 16 male patients had a total of 224 acute episodes over a median observation period of 8.3 years. The relapse rate was 2.6% per month, for women 2.4% and for men 3.5% per month. Since 2003, 17 patients with a first iTTP episode were treated with rituximab, whereas 28 were not. There was a trend towards lower relapse rates after rituximab treatment over the ensuing years. However, this was statistically not significant.
conclusionThis analysis does not show a significant reduction of acute iTTP relapses by rituximab given during an acute bout. Initial episodes are characterized by more severe clinical signs compared with the less severe relapses. Furthermore, men suffer significantly more frequent and considerably more serious acute relapses.
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