ReviewJournal of blood medicine2018
Evans syndrome: clinical perspectives, biological insights and treatment modalities.
Review in Journal of blood medicine, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 60 papers.
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
60 citing papers in PubMed, 118 citations in OpenAlex.
- Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.Pediatric reports · 2026Article
- From Hemolysis to Lupus: A Case of Evans Syndrome Revealing Systemic Autoimmunity.Clinical case reports · 2026Article
- Unmasking Evans Syndrome: A Case of New Onset Anemia and Thrombocytopenia in a Pregnant Patient With Systemic Lupus Erythematosus.Case reports in hematology · 2026Article
- Predictive factors for severity and poor treatment response in children with Evans syndrome: A retrospective cohort study.Frontiers in immunology · 2026Article
- Community-acquired Acinetobacter pneumonia associated with Evans syndrome: a case report.Annals of medicine and surgery (2012) · 2025Article
- 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
- Exacerbation of Evans syndrome in vaccinated pregnant woman with mild COVID-19 infection.AJOG global reports · 2025Article
- Article
- 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
- New-onset hematologic disorders following COVID-19 vaccination: a systematic review.Clinical and experimental vaccine research · 2025Article
- Thrombotic Thrombocytopenic Purpura and Evans Syndrome: Validating and Exploring 20 Years of Routine Hospital Care.Journal of blood medicine · 2025Article
- Clinical and laboratory profile of patients with positive direct antiglobulin test attended at a university hospital in Goias, Brazil.Hematology, transfusion and cell therapy · 2024Article
- Differential diagnosis of Guillain-Barré syndrome: steroid-responsive radiculopathy in Evans syndrome.Neurological research and practice · 2024Article
- Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.Cureus · 2024Article
- Recurrent Infection in a Young Female Patient Recently Diagnosed With Primary Evans Syndrome Without Neutropenia.Journal of hematology · 2024Article
- Splenectomy reappraised: Bridging the gap in immune cytopenia treatment paradigms.Journal of internal medicine · 2024Article
- The Successful Management of Stroke in Evans Syndrome by Anticoagulation with Warfarin, Intravenous Immunoglobulin (IVIG), and High-Dose Corticosteroid.European journal of case reports in internal medicine · 2024Article
- T-large granular lymphocyte frequencies and correlates in disease states detected by multiparameter flow cytometry in pediatric and young adult population.Annals of hematology · 2024Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person's lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Recent molecular theories explaining the physiopathology of ES include deficiencies of CTLA-4, LRBA, TPP2 and a decreased CD4/CD8 ratio. As in other autoimmune cytopenias, there is no established evidence-based treatment and steroids are the first-line therapy, with intravenous immunoglobulin administered as a life-saving resource in cases of severe immune thrombocytopenic purpura manifestations. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful, although it is necessary to consider its potential serious adverse effects. In conclusion, ES is a disease with a heterogeneous course that remains challenging to patients and physicians, with prospective clinical trials needed to explore potential targeted therapy to achieve an improved long-term response or even a cure.
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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.