SynthesisOrphanet journal of rare diseases2019
Incidence of acquired thrombotic thrombocytopenic purpura in Germany: a hospital level study.
Synthesis in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 44 citations in OpenAlex.
- Epidemiology of immune-mediated thrombotic thrombocytopenic purpura in Germany.Research and practice in thrombosis and haemostasis · 2026Article
- Current standards and novel concepts in thrombotic thrombocytopenic purpura.Frontiers in medicine · 2026Review
- Common features of rare disease patients in the emergency department: a systematised literature review.Orphanet journal of rare diseases · 2025Review
- Thrombotic thrombocytopenic purpura: early diagnosis and effective treatment in 2025.Intensive care medicine · 2025Review
- Article
- Use and application of geographical restrictions in systematic reviews with the aim of including studies about Germany: An update of a methodological review.Health information and libraries journal · 2024Review
- Real-World Data Analysis of Patients Affected by Immune-Mediated Thrombotic Thrombocytopenic Purpura in Italy.Journal of clinical medicine · 2024Article
- A Systematic Review of the Epidemiology and Disease Burden of Congenital and Immune-Mediated Thrombotic Thrombocytopenic Purpura.Journal of blood medicine · 2024Review
- Real-world effectiveness of caplacizumab vs the standard of care in immune thrombotic thrombocytopenic purpura.Blood advances · 2022Article
- Relapse of immune-mediated thrombotic thrombocytopenic purpura following mRNA COVID-19 vaccination: a prospective cohort study.Haematologica · 2022Article
- Efficacy and safety of azathioprine during remission of immune-mediated thrombotic thrombocytopenic purpura.Blood advances · 2022Article
- Incidence of thrombotic microangiopathies in Quebec: insight from a laboratory centralizing ADAMTS-13 testing.Orphanet journal of rare diseases · 2022Article
- Epidemiology, treatment patterns, clinical outcomes, and disease burden among patients with immune-mediated thrombotic thrombocytopenic purpura in the United States.Research and practice in thrombosis and haemostasis · 2022Article
- The Challenges in Diagnosis and Management of Acquired Thrombotic Thrombocytopenic Purpura: Consensus Report from Three Gulf Countries.Oman medical journal · 2022Review
- A Race against the Clock: A Case Report and Literature Review Concerning the Importance of ADAMTS13 Testing in Diagnosis and Management of Thrombotic Thrombocytopenic Purpura during Pregnancy.Diagnostics (Basel, Switzerland) · 2022Article
- Lombardy diagnostic and therapeutic network of thrombotic microangiopathy.Orphanet journal of rare diseases · 2022Article
- Immune-mediated thrombotic thrombocytopenic purpura following administration of Pfizer-BioNTech COVID-19 vaccine.Haematologica · 2022Article
- First diagnosis of thrombotic thrombocytopenic purpura after SARS-CoV-2 vaccine - case report.BMC nephrology · 2021Article
- Acute myocardial infarction and extensive systemic thrombosis in thrombotic thrombocytopenic purpura: A case report and review of literature.World journal of clinical cases · 2021Article
- First report of a de novo iTTP episode associated with an mRNA-based anti-COVID-19 vaccination.Journal of thrombosis and haemostasis : JTH · 2021Article
Corrections and comments
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Authors and funding
13 authors at 10 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAcquired thrombotic thrombocytopenic Purpura (aTTP) is a life-threatening ultra-orphan disease with a reported annual incidence between 1.5 and 6.0 cases per million in Europe and mainly affecting otherwise young and healthy adults aged 40 years on average. The goal of this study was to assess the incidence of aTTP in Germany.
methodsA systematic review was performed to determine the published evidence on the aTTP epidemiology in Germany. To obtain additional evidence on the proportion of aTTP cases within the national Thrombotic Microangiopathy (TMA) population a hospital-level study was performed, using a retrospective data collection approach. Diagnosis of aTTP was confirmed if ADAMTS13 level were < 10% and/or the medical records explicitly mentioned aTTP diagnosis. The aggregated hospital data were then projected to the national level using logistic regression techniques.
resultsThe systematic literature search did not provide incidence estimates of aTTP in Germany. Eight centers (≈27% of the top 30 TMA hospitals) delivered data according to a predefined data collection form. On average (year 2014-2016) a total number of 172 aTTP episodes per year was projected (95% confidence interval [95%CI]: 132-212). The majority were newly diagnosed aTTP cases (n = 121; 95%CI: 105-129), and 51 were recurrent aTTP cases (95%CI: 27-84). The average annual projected incidence (year 2014-2016) of aTTP episodes was 2.10 per million inhabitants in Germany (95%CI: 1.60-2.58).
conclusionsThe determined annual incidence of newly diagnosed aTTP cases and the overall annual incidence of aTTP episodes in Germany confirm the ultra-orphan character of aTTP. An external validation against international registries (France, UK and USA) shows that our findings are quite comparable with those international incidence rates.
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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.