ArticleFrontiers in pediatrics2019
Evans Syndrome in Childhood: Long Term Follow-Up and the Evolution in Primary Immunodeficiency or Rheumatological Disease.
Article in Frontiers in pediatrics, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 3 of them syntheses that pooled it.
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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.
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Who cites it
22 citing papers in PubMed, 3 syntheses or guidelines pooled it, 32 citations in OpenAlex.
- Red flags to suspect inborn errors of immunity in patients with autoimmune diseasesBiomedica : revista del Instituto Nacional de Salud · 2024Pooled it
- Prevalence of Immunological Defects in a Cohort of 97 Rubinstein-Taybi Syndrome Patients.Journal of clinical immunology · 2020Pooled it
- Combination of Evans syndrome and COVID-19: a systematic review of reported cases.Blood transfusion = Trasfusione del sanguePooled it
- Autoimmune Cytopenias and Dysregulated Immunophenotype Act as Warning Signs of Inborn Errors of Immunity: Results From a Prospective Study.Frontiers in immunology · 2021Trial
- From Hemolysis to Lupus: A Case of Evans Syndrome Revealing Systemic Autoimmunity.Clinical case reports · 2026Article
- The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency.International journal of molecular sciences · 2025Review
- Case Report: Early-onset or recalcitrant cytopenias as presenting manifestations of activated PI3Kδ syndrome.Frontiers in pediatrics · 2024Article
- Immune-Complex Mediated Mesangial Proliferative Glomerulonephritis with Full-House Pattern Observed During Treatment of Immune Thrombocytopenic Purpura.Clinical medicine insights. Case reports · 2024Article
- Extended List of Warning Signs in Qualification to Diagnosis and Treatment of Inborn Errors of Immunity in Children and Young Adults.Journal of clinical medicine · 2023Article
- Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy.Journal of endocrinological investigation · 2023Review
- Immune dysregulation in Kabuki syndrome: a case report of Evans syndrome and hypogammaglobulinemia.Frontiers in pediatrics · 2023Article
- Comprehensive phenotyping of human peripheral blood B lymphocytes in pathological conditions.Cytometry. Part A : the journal of the International Society for Analytical Cytology · 2022Article
- Article
- Underlying Inborn Errors of Immunity in Patients With Evans Syndrome and Multilineage Cytopenias: A Single-Centre Analysis.Frontiers in immunology · 2022Article
- Response to rituximab in children and adults with immune thrombocytopenia (ITP).Research and practice in thrombosis and haemostasis · 2021Article
- Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed LymphomaTurkish journal of haematology : official journal of Turkish Society of Haematology · 2021Article
- Immune cytopenias as a continuum in inborn errors of immunity: An in-depth clinical and immunological exploration.Immunity, inflammation and disease · 2021Article
- Autoimmune Hemolytic Anemia in the Pediatric Setting.Journal of clinical medicine · 2021Review
- Case Report: Post-Partum Complications ofFrontiers in pediatrics · 2021Article
- Screening for Genetic Mutations for the Early Diagnosis of Common Variable Immunodeficiency in Children With Refractory Immune Thrombocytopenia: A Retrospective Data Analysis From a Tertiary Children's Center.Frontiers in pediatrics · 2020Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Evans syndrome (ES) is a rare but challenging condition, characterized by recurrent and refractory cytopenia episodes. Recent discoveries highlighted that an appropriate diagnostic workup is fundamental to identify an underlying immune dysregulation such as primary immunodeficiencies or a rheumatological disease. We hereby describe clinical features and laboratory results of 12 pediatric patients affected by ES referred to the Pediatric Onco-Hematology Unit of Bologna. Patients experienced a median of four acute episodes of cytopenia with 9 years as median age at the onset of symptoms. In 8/12 (67%) patients an underlying etiology, primary immunodeficiencies, or rheumatological disease was identified. In 4/12 children, other immune manifestations were associated (Thyroiditis, Celiac disease, Psoriasis, Vitiligo, Myositis, Membranoproliferative Glomerulonephritis). ES remained the primary diagnosis in four patients (33%). At a median follow-up time of 4 years, 5/12 (42%) patients revealed a chronic ITP, partially responsive to second line therapy. Immunoglobulin Replacement Therapy (IRT) was effective with a good hematological values control in three patients with a secondary ES (ALPS, CVID, and a patient with Rubinstein Taybi Syndrome and a progressive severe B cell deficiency with hypogammaglobulinemia). Our experience highlights that, in pediatric patients, ES is often only the first manifestation of an immunological or rheumatological disease, especially when cytopenias are persistent or resistant to therapy, with an early-onset or when are associated with lymphadenopathy.
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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.