ArticleHaematologica2022
Long term follow-up of pediatric-onset Evans syndrome: broad immunopathological manifestations and high treatment burden.
Article in Haematologica, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05937828 ("French National Cohort of Patients With Pediatric-onset of Autoimmune Cytopenia), which is not on this map. Cited by 15 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.
"French National Cohort of Patients With Pediatric-onset of Autoimmune Cytopenia (OBS'CEREVANCE Cohort)"
Who cites it
15 citing papers in PubMed, 1 synthesis or guideline pooled it, 25 citations in OpenAlex.
- Combination of Evans syndrome and COVID-19: a systematic review of reported cases.Blood transfusion = Trasfusione del sanguePooled it
- Pediatric autoimmune hemolytic anemia is associated with a high incidence of underlying immune disorders.Blood advances · 2026Article
- Chronic autoimmune cytopenias in pediatric age group: Clinical features and outcome.Hematology, transfusion and cell therapy · 2026Article
- Paediatric-onset autoimmune cytopenia: How can we reduce the long-term mortality?British journal of haematology · 2026Article
- Increased percentages of circulating T follicular helper cells associate with disease subtype and activity in pediatric immune cytopenias.Haematologica · 2026Article
- Genetic testing guides therapy in children with refractory cytopenias.Haematologica · 2026Article
- Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.Pediatric reports · 2026Article
- Azathioprine or mycophenolate mofetil for pediatric autoimmune cytopenia: a propensity score-matched study.Blood advances · 2026Article
- Childhood isolated C3d-positive AIHA: favorable prognosis but rare relapse or associated immunodeficiency.Blood advances · 2026Article
- Antiphospholipid Syndrome Coexisting With Evans Syndrome and SCL-70 Antibody Positivity: A Case Report.Clinical case reports · 2026Article
- Predictive factors for severity and poor treatment response in children with Evans syndrome: A retrospective cohort study.Frontiers in immunology · 2026Article
- Review
- Impact of Thrombopoietin Receptor Agonists on Pathophysiology of Pediatric Immune Thrombocytopenia.Current issues in molecular biology · 2025Review
- Evaluating the prevalence of inborn errors of immunity in adults with chronic immune thrombocytopenia or Evans syndrome.Blood advances · 2023Article
- Evans syndrome in adults: an observational multicenter study.Blood advances · 2021Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
31 authors at 20 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pediatric-onset Evans syndrome (pES) is defined by both immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) before the age of 18 years. There have been no comprehensive long-term studies of this rare disease, which can be associated to various immunopathological manifestations (IM). We report outcomes of the 151 patients with pES and more than 5 years of follow-up from the nationwide French prospective OBS'CEREVANCE cohort. Median age at final follow-up was 18.5 years (range, 6.8-50.0 years) and the median follow-up period was 11.3 years (range, 5.1-38.0 years). At 10 years, ITP and AIHA were in sustained complete remission in 54.5% and 78.4% of patients, respectively. The frequency and number of clinical and biological IM increased with age: at the age of 20 years, 74% had at least one clinical IM (cIM). A wide range of cIM occurred, mainly lymphoproliferation, dermatological, gastrointestinal/hepatic and pneumological IM. The number of cIM was associated with a subsequent increase in the number of second-line treatments received (other than steroids and immunoglobulins; hazard ratio 1.4, 95% Confidence Interval: 1.15-1.60, P=0.0002, Cox proportional hazards method). Survival at 15 years after diagnosis was 84%. Death occurred at a median age of 18 years (range, 1.7-31.5 years), and the most frequent cause was infection. The number of second-line treatments and severe/recurrent infections were independently associated with mortality. In conclusion, long-term outcomes of pES showed remission of cytopenias but frequent IM linked to high second-line treatment burden. Mortality was associated to drugs and/or underlying immunodeficiencies, and adolescents-young adults are a high-risk subgroup.
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Registered trials
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.