ArticleBlood advances2021
Evans syndrome in adults: an observational multicenter study.
Article in Blood advances, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers, 1 of them a synthesis that pooled it.
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Who cites it
43 citing papers in PubMed, 1 synthesis or guideline pooled it, 68 citations in OpenAlex.
- Combination of Evans syndrome and COVID-19: a systematic review of reported cases.Blood transfusion = Trasfusione del sanguePooled it
- Two cases of durvalumab-induced Evans syndrome in biliary tract cancer.Clinical journal of gastroenterology · 2026Article
- Targeting CD38 Across Autoimmune and Plasma Cell-Driven Immune-Mediated Diseases: Rationale, Clinical Evidence, and Emerging Therapeutic Landscape.BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy · 2026Review
- Single low-dose daratumumab enhances rituximab efficacy for sustained remission in treatment-refractory autoimmune cytopenias.Annals of hematology · 2026Article
- Perioperative management of Evans Syndrome for free tissue transfer: Case report and recommendations.JPRAS open · 2026Article
- Thrombophilia screening in patients with autoimmune hemolytic anemia: a single-center analysis.Haematologica · 2026Article
- Comparison of Evans syndrome and isolated autoimmune cytopenias in SLE: a retrospective study.Lupus science & medicine · 2026Article
- [Treatment practice and outcomes in 125 patients with autoimmune hemolytic anemia: a single-center retrospective analysis].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2026Article
- Multifactorial thrombotic risk in Evans syndrome: a case report and review of the literature.Thrombosis journal · 2026Article
- Mutations in histone lysine methyltransferase genes are associated with autoimmune cytopenias: a single-center study.Blood vessels, thrombosis & hemostasis · 2026Article
- Real-World Use of Avatrombopag in Children and Adolescents With Immune Thrombocytopenia.Pediatric blood & cancer · 2026Article
- Management of autoimmune hemolytic anemia.Hematology. American Society of Hematology. Education Program · 2025Review
- Severe relapse of Evans syndrome in an adult patient with treatment resistance and fatal outcome: A case report.The Journal of international medical research · 2025Article
- Article
- Community-acquired Acinetobacter pneumonia associated with Evans syndrome: a case report.Annals of medicine and surgery (2012) · 2025Article
- Rituximab as an Effective Treatment for New-onset Evans Syndrome and Systemic Lupus Erythematosus with Lupus Nephritis.Internal medicine (Tokyo, Japan) · 2025Article
- Evans Syndrome and COVID-19 Infection or Vaccination: A Systematic Review of Case Reports.Journal of hematology · 2025Review
- Evans Syndrome: Analysis of 1255 Adult Cases in US Hospitals, Revealing Demographics, Associations, and Outcomes.International journal of hematology-oncology and stem cell research · 2025Article
- Article
- Thrombotic storm with antiphospholipid syndrome in the setting of relapsing Evans syndrome and systemic lupus erythematosus: a case report.AME case reports · 2025Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
21 authors at 13 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Evans syndrome (ES) is a rare condition, defined as the presence of 2 autoimmune cytopenias, most frequently autoimmune hemolytic anemia and immune thrombocytopenia (ITP) and rarely autoimmune neutropenia. ES can be classified as primary or secondary to various conditions, including lymphoproliferative disorders, other systemic autoimmune diseases, and primary immunodeficiencies, particularly in children. In adult ES, little is known about clinical features, disease associations, and outcomes. In this retrospective international study, we analyzed 116 adult patients followed at 13 European tertiary centers, focusing on treatment requirements, occurrence of complications, and death. ES was secondary to or associated with underlying conditions in 24 cases (21%), mainly other autoimmune diseases and hematologic neoplasms. Bleeding occurred in 42% of patients, mainly low grade and at ITP onset. Almost all patients received first-line treatment (steroids with or without intravenous immunoglobulin), and 23% needed early additional therapy for primary refractoriness. Additional therapy lines included rituximab, splenectomy, immunosuppressants, thrombopoietin receptor agonists, and others, with response rates >80%. However, a remarkable number of relapses occurred, requiring ≥3 therapy lines in 54% of cases. Infections and thrombotic complications occurred in 33% and 21% of patients, respectively, mainly grade ≥3, and correlated with the number of therapy lines. In addition to age, other factors negatively affecting survival were severe anemia at onset and occurrence of relapse, infection, and thrombosis. These data show that adult ES is often severe and marked by a relapsing clinical course and potentially fatal complications, pinpointing the need for high clinical awareness, prompt therapy, and anti-infectious/anti-thrombotic prophylaxis.
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Registered trials
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