ArticleBlood2022
T-follicular helper cell expansion and chronic T-cell activation are characteristic immune anomalies in Evans syndrome.
Article in Blood, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 2 of them syntheses that pooled it.
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Who cites it
24 citing papers in PubMed, 2 syntheses or guidelines pooled it, 33 citations in OpenAlex.
- Red flags to suspect inborn errors of immunity in patients with autoimmune diseasesBiomedica : revista del Instituto Nacional de Salud · 2024Pooled it
- Combination of Evans syndrome and COVID-19: a systematic review of reported cases.Blood transfusion = Trasfusione del sanguePooled it
- Circulating T follicular helper cells as emerging biomarkers in pediatric Evans syndrome.Haematologica · 2026Article
- Increased percentages of circulating T follicular helper cells associate with disease subtype and activity in pediatric immune cytopenias.Haematologica · 2026Article
- Progressive cerebral infarction as the presenting feature of ANCA-associated vasculitis with concurrent Evans syndrome: a case report and literature review.Frontiers in immunology · 2026Review
- Refractory immune cytopenia successfully treated with mycophenolate mofetil in four adolescents with del22q11.2 syndrome.Frontiers in immunology · 2026Article
- T follicular helper cells in primary immune regulatory disorders.The Journal of allergy and clinical immunology · 2025Review
- Double Trouble: The First Reported Case of Evans Syndrome Following RSV Vaccination.Hematology reports · 2025Article
- T-cell and autoantibody profiling for primary immune regulatory disorders.The Journal of allergy and clinical immunology · 2025Article
- Review
- Comprehensive RNA-seq analysis of benign prostatic hyperplasia (BPH) in rats exposed to testosterone and estradiol.Scientific reports · 2025Article
- Building alliances for early detection of immunodeficiencies: from primary care to hematology.Frontiers in immunology · 2025Review
- Beneath the surface in autoimmune hemolytic anemia: pathogenetic networks, therapeutic advancements and open questions.Frontiers in immunology · 2025Review
- Role of circulating T follicular helper subsets following Ty21a immunization and oral challenge with wild typeFrontiers in immunology · 2024Article
- Frequency of HLA-DRThe Journal of allergy and clinical immunology · 2024Article
- Evaluating the prevalence of inborn errors of immunity in adults with chronic immune thrombocytopenia or Evans syndrome.Blood advances · 2023Article
- Article
- Clinical utility of measuring CD4Journal of autoimmunity · 2023Article
- Article
- Evans Syndrome as a Possible Complication of Brentuximab Vedotin Therapy for Peripheral T Cell Lymphoma.Hematology reports · 2023Article
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21 authors at 5 institutions in 1 country.
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Abstract
Pediatric Evans syndrome (pES) is increasingly identified as the presenting manifestation of several inborn errors of immunity. Despite an improved understanding of genetic defects in pES, the underlying immunobiology of pES is poorly defined, and characteristic diagnostic immune parameters are lacking. We describe the immune characteristics of 24 patients with pES and compared them with 22 patients with chronic immune thrombocytopenia (cITP) and 24 healthy controls (HCs). Compared with patients with cITP and HC, patients with pES had increased circulating T-follicular helper cells (cTfh), increased T-cell activation, and decreased naïve CD4+ T cells for age. Despite normal or high immunoglobulin G (IgG) in most pES at presentation, class-switched memory B cells were decreased. Within the cTfh subset, we noted features of postactivation exhaustion with upregulation of several canonical checkpoint inhibitors. T-cell receptor β chain (TCR-β) repertoire analysis of cTfh cells revealed increased oligoclonality in patients with pES compared with HCs. Among patients with pES, those without a known gene defect had a similar characteristic immune abnormality as patients with defined genetic defects. Similarly, patients with pES with normal IgG had similar T-cell abnormalities as patients with low IgG. Because genetic defects have been identified in less than half of patients with pES, our findings of similar immune abnormalities across all patients with pES help establish a common characteristic immunopathology in pES, irrespective of the underlying genetic etiology.
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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.