A Síndrome de Evans é uma doença crônica e rara do sangue, caracterizada pela ocorrência conjunta ou em sequência de: * **Anemia Hemolítica Autoimune (AIHA):** uma condição em que o próprio sistema de defesa do corpo (os anticorpos) ataca os glóbulos vermelhos (células vermelhas do sangue), causando anemia que pode ser de leve a grave; * **Púrpura Trombocitopênica Imune (PTI):** um problema na coagulação do sangue em que o próprio sistema de defesa (os anticorpos) ataca as plaquetas, provocando episódios de sangramento; * e, ocasionalmente, **neutropenia autoimune**. Tudo isso acontece sem uma causa primária conhecida.
Introdução
O que você precisa saber de cara
A Síndrome de Evans é uma doença crônica e rara do sangue, caracterizada pela ocorrência conjunta ou em sequência de: * **Anemia Hemolítica Autoimune (AIHA):** uma condição em que o próprio sistema de defesa do corpo (os anticorpos) ataca os glóbulos vermelhos (células vermelhas do sangue), causando anemia que pode ser de leve a grave; * **Púrpura Trombocitopênica Imune (PTI):** um problema na coagulação do sangue em que o próprio sistema de defesa (os anticorpos) ataca as plaquetas, provocando episódios de sangramento; * e, ocasionalmente, **neutropenia autoimune**. Tudo isso acontece sem uma causa primária conhecida.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 7 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome Evans
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Publicações mais relevantes
Investigating Biomarkers for Inborn Errors of Immunity in a Prospective Study of Patients With Autoimmune Cytopenia.
In a prospective cohort from the Tampa Bay region (2016-2020), patients with autoimmune cytopenia (AIC) were evaluated to identify cellular and serum biomarkers that distinguish those with underlying inborn errors of immunity (IEI). Clinical phenotype and genetic causes of IEI were assessed using targeted panel-based sequencing. Unique lymphocyte subsets, including activated naïve and transitional B cells, CD19hiCD21lo B cells, follicular helper T (TFH) cells, regulatory T (Treg) cells, and TCRαβ+CD4-CD8- double-negative T cells (DNTαβ), were assessed by flow cytometry. Serum levels of lipopolysaccharide (LPS), B-cell activating factor (BAFF), and soluble IL-2 receptor (sIL2R) were quantified by ELISA. Among 104 AIC patients, 53 (51%) showed evidence of IEI, including 27 (26%) with monogenic disorders-most commonly partial DiGeorge syndrome (pDGS), followed by variants in NFKB1, CTLA4, and FAS. The prevalence of IEI was highest in autoimmune hemolytic anemia (AIHA) (62.5%) and Evans syndrome (61.5%). Low levels of IgG, IgA, and IgM, as well as reduced percentages of naïve CD4+ and CD8+ T cells, were significantly associated with increased odds of IEI. In AIC-IEI patients, transitional B cells, CD19hiCD21lo B cells, and TFH cells were expanded, accompanied by elevated serum levels of BAFF and sIL2R. Quantitative immunoglobulin levels and naïve T cells remain valuable indicators of IEI in AIC. Our findings highlight the diagnostic value of emerging cellular and serum biomarkers in identifying IEI, including dysregulation of early B-cell subsets (transitional B cells and CD19hiCD21lo B cells), expansion of TFH cells, and elevated levels of BAFF and sIL2R.
Pediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.
Evans syndrome (ES) is a rare immune-mediated disorder involving two or more cytopenias, including immune thrombocytopenia (ITP), autoimmune hemolytic anemia, and/or immune neutropenia. ES may occur secondary to another condition or be idiopathic. While consensus recommendations exist for adults, there is no standardized diagnostic approach for pediatric Evans syndrome (pES). This study aimed to describe typical diagnostic evaluations conducted by clinicians caring for pES patients. A cross-sectional survey of the Pediatric ITP Consortium of North America (ICON) assessed typical diagnostic workup for pES, the influence of clinical features on testing, evaluation for underlying disorders, including immune defects and autoimmune disease, subspecialty involvement, and genetic testing practices. Sixty percent (28/47) of respondents reported performing the same evaluation for all pES patients. There was no consensus on specific diagnostic tests. Providers consistently evaluated for autoimmune conditions, but varied in testing for inborn errors of immunity (IEI). Rheumatology and immunology were most often consulted. Most respondents (85%, n = 40) obtained genetic testing through commercial laboratories, frequently encountering insurance-related barriers. Even among experts, diagnostic approaches to pES vary widely. Standardized frameworks are needed to guide comprehensive evaluation for this complex disorder.
Azathioprine or mycophenolate mofetil for pediatric autoimmune cytopenia: a propensity score-matched study.
Data on the immunosuppressants azathioprine (AZA) and mycophenolate mofetil (MMF) in autoimmune cytopenia (AIC) are limited, and no direct comparison exists. We analyzed the failure-free survival (FFS; time from AZA/MMF initiation to another non-first-line treatment, or death) of both treatments in the prospective nationwide pediatric OBS'CEREVANCE cohort. We included 343 patients (chronic immune thrombocytopenia, n = 161; autoimmune hemolytic anemia, n = 74; Evans syndrome, n = 108). They received AZA (n = 276) or MMF (n = 104; 37 sequentially received both) as monotherapy for a median duration of 11.3 (range, 0.01-149.0) months. Older age was associated with higher FFS for AZA, whereas secondary AIC was associated with higher FFS for MMF. AIC type had no effect. In a propensity score (PS)-matched cohort, AZA and MMF showed similar FFS (adjusted hazard ratio, 0.91; 95% confidence interval, 0.54-1.52; P = .71), with 1-year FFS rates of 73% and 76%, respectively. In subgroup analyses, AZA was associated with higher FFS in PS-matched patients diagnosed at an age of ≥10 years, whereas MMF was associated with higher FFS in PS-matched patients diagnosed at an age of <10 years and in those with confirmed secondary AIC (although with suboptimal matching). Rates of grade ≥3 infection were similar between the 2 drugs, at ∼2% new cases per year. In summary, AZA and MMF demonstrated comparable overall FFS and infection risk. However, our data suggest that AZA may be more beneficial in children diagnosed at an age of ≥10 years, whereas MMF may be more beneficial in those diagnosed at an age of <10 years and possibly in patients with secondary AIC.
Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.
Background: Pediatric-onset Evans syndrome (pES) is a rare autoimmune disorder defined by the coexistence or sequential development of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA), frequently accompanied by autoimmune neutropenia (AIN) and characterized by a relapsing, multilineage course. Increasing evidence suggests that pES may represent a broader immune dysregulation phenotype rather than an isolated hematologic disorder. Methods: We conducted a retrospective, single-center study of children diagnosed with pES and followed for up to 13 years at a tertiary referral center. Clinical data regarding hematologic evolution, extra-hematological immunopathological manifestations, treatment requirements, infectious complications, and genetic findings were analyzed descriptively. Results: Six children (4 males) were included, with a median age at first cytopenia of 7 years (range 3-15) and a median follow-up of 8 years (range 1-13). ITP preceded AIHA in 3/6 patients (50%), one patient (16.7%) developed AIHA first, and two (33.3%) showed partial or evolving multilineage disease with DAT positivity prior to overt hemolysis. AIN occurred in 3/6 patients (50%). Extra-hematological immunopathological manifestations occurred in 5/6 patients (83.3%), with two (33.3%) developing more than one. Second-line therapy was required in 3/6 patients (50%). Infectious episodes occurred in 83.3% of patients, predominantly viral or mild bacterial infections, with no life-threatening events. Whole-exome sequencing performed in three patients identified a heterozygous TNFAIP3 variant of uncertain significance in one case; no pathogenic variants were detected. Conclusions: pES demonstrates clinical heterogeneity, frequent multilineage cytopenia, and substantial extra-hematological immune involvement. Multisystem manifestations may be associated with increased treatment burden. Long-term multidisciplinary monitoring and cautious interpretation of genetic findings are essential for individualized pediatric care.
A multicentre analysis of efficacy, safety and molecular response correlates of fostamatinib in warm autoimmune haemolytic anaemia and Evans syndrome.
Fostamatinib had 46% durable response, with 73% steroid reduction, in this multicentre retrospective study of refractory wAIHA/ES. Hypertension, gastrointestinal (GI) distress and neutropenia occurred in 23%. Only one patient required drug discontinuation and one patient dose reduction. In an eight-patient subset, migration inhibitory factor (MIF) levels, naïve (CD62Lhi) T-cell and HLA-DRhi monocyte subsets each correlated with treatment response.
Publicações recentes
Alcohol-Induced Acute Pancreatitis Complicated by Severe Direct Antiglobulin Test (DAT)-Negative Hemolytic Anemia and Thrombocytopenia: A Suspected Evans Syndrome-Like Immune Cytopenia.
Comparison of Evans syndrome and isolated autoimmune cytopenias in SLE: a retrospective study.
Anti-CD19 chimeric antigen receptor T cells in Evans syndrome of systemic lupus erythematosus.
Combined mesenchymal stem cells and telitacicept therapy for Evans syndrome refractory to various immunosuppressive: a case report.
Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.
📚 EuropePMC516 artigos no totalmostrando 195
Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.
Pediatric reportsA multicentre analysis of efficacy, safety and molecular response correlates of fostamatinib in warm autoimmune haemolytic anaemia and Evans syndrome.
British journal of haematologyCase Report: Native aortic valve Listeria monocytogenes endocarditis in an adult with Evans syndrome.
Frontiers in immunologyPredictive factors for severity and poor treatment response in children with Evans syndrome: A retrospective cohort study.
Frontiers in immunologyCirculating T follicular helper cells as emerging biomarkers in pediatric Evans syndrome.
HaematologicaFrom Hemolysis to Lupus: A Case of Evans Syndrome Revealing Systemic Autoimmunity.
Clinical case reportsMultifactorial thrombotic risk in Evans syndrome: a case report and review of the literature.
Thrombosis journalMultisystem Complications in Postpartum-Onset Evans Syndrome: A Case Report.
CureusImmune-Mediated Multilineage Cytopenias in Thymoma: A Retrospective Case Series.
Thoracic cancerInvestigating Biomarkers for Inborn Errors of Immunity in a Prospective Study of Patients With Autoimmune Cytopenia.
Pediatric blood & cancerPediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.
Pediatric blood & cancerIncidence, Risk Factors, and Treatment of Autoimmune Cytopenia Following Pediatric Allogeneic Hematopoietic Stem Cell Transplantation.
Journal of transplantationAntiphospholipid Syndrome Coexisting With Evans Syndrome and SCL-70 Antibody Positivity: A Case Report.
Clinical case reportsDouble Trouble: The First Reported Case of Evans Syndrome Following RSV Vaccination.
Hematology reportsWhat We Miss in Patients With Persistent Thrombocytopenia During the Dengue Season.
CureusSevere relapse of Evans syndrome in an adult patient with treatment resistance and fatal outcome: A case report.
The Journal of international medical researchCommunity-acquired Acinetobacter pneumonia associated with Evans syndrome: a case report.
Annals of medicine and surgery (2012)Exacerbation of Evans syndrome in vaccinated pregnant woman with mild COVID-19 infection.
AJOG global reportsSuccessful Treatment of Evans Syndrome With efgartigimod: A Case Report.
American journal of therapeuticsHematological toxicity of immune checkpoint inhibitors: a pharmacovigilance study.
BMC cancerTransient Haemolytic Anaemia and Thrombocytopenia in a Healthy Young Adult Following Influenza a Infection.
European journal of case reports in internal medicineA New Variant in CTLA4 Highlights the Heterogeneous Phenotype of CTLA4 Haploinsufficiency.
Journal of clinical immunologyEvidence that mycophenolate mofetil is a safe and effective immunosuppressive in the treatment of autoimmune hepatitis and Evans syndrome: Case report.
International journal of clinical pharmacology and therapeuticsIncreased percentages of circulating T follicular helper cells associate with disease subtype and activity in pediatric immune cytopenias.
HaematologicaAzathioprine or mycophenolate mofetil for pediatric autoimmune cytopenia: a propensity score-matched study.
Blood advancesEvans Syndrome in a 35-Year-Old Male: Diagnostic Challenges and Multimodal Management.
CureusOxaliplatin-induced type II hypersensitivity in colorectal cancer: a cohort study on clinical presentation, diagnosis, and management.
Frontiers in pharmacology[Efficacy and Safety of Zanubrutinib in the Treatment of Autoimmune Cytopenia Secondary to Indolent B-Cell Lymphoma].
Zhongguo shi yan xue ye xue za zhiEvans syndrome in pregnancy and the postpartum period: A retrospective cohort study.
British journal of haematologyEffectiveness of sirolimus for early on-set autoimmune cytopenias of autoimmune lymphoproliferative immunodeficiencies.
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and ImmunologyAcute Hemolytic Anemia and Immune Thrombocytopenia: A Rare Case of Parvovirus B19-Induced Evans Syndrome.
CureusSevere hemolysis flare of refractory autoimmune hemolytic anemia with positive complement component C3d responsive to Iptacopan with cyclophosphamide and prednisone: a case report.
Hematology (Amsterdam, Netherlands)Evans Syndrome: Analysis of 1255 Adult Cases in US Hospitals, Revealing Demographics, Associations, and Outcomes.
International journal of hematology-oncology and stem cell researchAutoimmune/Inflammatory Syndrome Induced by Adjuvants (ASIA) in Breast Implants: A Case Report.
CureusThrombotic storm with antiphospholipid syndrome in the setting of relapsing Evans syndrome and systemic lupus erythematosus: a case report.
AME case reportsEvans syndrome revisited.
Blood reviewsPrimary antiphospholipid syndrome in pediatrics: New criteria, new opportunities.
LupusWhole Exome Sequencing of Adult Patients With Evans Syndrome Reveals Pathogenic Variants Associated With Autoimmunity.
American journal of hematologyImmunotherapy-associated hemostatic abnormalities: bleeding and thrombotic complications.
Annals of hematologyThrombotic Thrombocytopenic Purpura and Evans Syndrome: Validating and Exploring 20 Years of Routine Hospital Care.
Journal of blood medicineEvans Syndrome and COVID-19 Infection or Vaccination: A Systematic Review of Case Reports.
Journal of hematologyA Room for Long-Lived Plasma Cell Contribution in Immune Cytopenias?
CancersA Case of Systemic Lupus Erythematosus Complicated by Secondary Evans Syndrome.
CureusLipopolysaccharide-responsive and beige-like anchor protein (LRBA) functional deficiency caused by biallelic LRBA missense variants characterized by Evans syndrome or colitis.
The Journal of allergy and clinical immunologyConcurrent pulmonary TB and histoplasmosis in an HIV-negative patient with Evans Syndrome.
IJTLD openRituximab as an Effective Treatment for New-onset Evans Syndrome and Systemic Lupus Erythematosus with Lupus Nephritis.
Internal medicine (Tokyo, Japan)Clinical Characteristics of Patients With Idiopathic Multicentric Castleman Disease Complicated by Autoimmune Hemolytic Anemia.
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeT and NK cell functionality in a patient harboring heterozygous novel BCL11B p.Asp632fsAla∗91 and STX11 p.R129P mutations.
HeliyonCombination of Evans syndrome and COVID-19: a systematic review of reported cases.
Blood transfusion = Trasfusione del sangueMixed histiocytic sarcoma in a Bernese Mountain Dog.
Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, IncSOCS1 deficiency-crossroads of autoimmunity and autoinflammation-two case reports.
Frontiers in pediatricsBudd-Chiari Syndrome as an Initial Manifestation of Incomplete Systemic Lupus Erythematosus.
European journal of case reports in internal medicineProlonged Severe CD4+ Lymphocytopenia and Hypogammaglobulinemia in Patients With Evans' Syndrome: A Case Report.
CureusCerebral aspergillosis in a patient with chronic lymphocytic leukaemia complicated by Evans syndrome.
BMJ case reportsEvans syndrome as a presentation in systemic lupus erythematous, coexisting with Hashimoto's thyroiditis and pernicious anemia: a case report.
Journal of medical case reportsPulmonary and Medullary Tuberculosis: An Uncommon Cause of Evans Syndrome in Adults.
CureusBeyond FAScinating: advances in diagnosis and management of autoimmune lymphoproliferative syndrome and activated PI3 kinase δ syndrome.
Hematology. American Society of Hematology. Education ProgramA rare paraneoplastic condition in Hodgkin lymphoma: Evans syndrome and literature review.
Northern clinics of Istanbul[Chinese expert consensus on the diagnosis and treatment of Evans syndrome (2024)].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiEvans syndrome in conjunction with rheumatoid arthritis and SLE: A unique case of autoimmune intersection.
Radiology case reportsRapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.
Allergologie selectGranulomatous Brain Involvement in Common Variable Immunodeficiency: A Case Report.
CureusDifferential diagnosis of Guillain-Barré syndrome: steroid-responsive radiculopathy in Evans syndrome.
Neurological research and practiceThrombotic thrombocytopenic purpura masquerading as Evans syndrome.
American journal of hematologyElective splenectomy for hematological diseases: a vanishing indication.
Surgical endoscopyDiagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.
CureusAssociation of paediatric autoimmune cytopenia and inflammatory bowel disease suggests a common genetic origin.
British journal of haematologySecondary Evans Syndrome Presenting With Lupus Anticoagulant.
CureusFamilial autoimmunity and risk of developing immune thrombocytopenia and Evans syndrome.
Pediatric blood & cancerExtremely Rare Case of Successful Treatment of Foot Ulcer Associated with Evans' Syndrome and Buerger's Disease.
Medicina (Kaunas, Lithuania)Navigating the conundrum of co-existing autoantibodies and alloantibodies in a case of Evans syndrome.
Laboratory medicineEvans syndrome: Disease awareness and clinical management in a nation-wide ITP-NET survey.
European journal of haematologyHaploinsufficiency in PTPN2 leads to early-onset systemic autoimmunity from Evans syndrome to lupus.
The Journal of experimental medicineEvans syndrome suggests disease progression in lung adenocarcinoma.
Respiratory medicine case reportsRecurrent Infection in a Young Female Patient Recently Diagnosed With Primary Evans Syndrome Without Neutropenia.
Journal of hematologyThe 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 medicineDiagnosis and management of Evans syndrome in adults: first consensus recommendations.
The Lancet. HaematologySpondyloenchondrodysplasia With Immune Dysregulation, but Without Skeletal Dysplasia, in a Six-Year-Old Boy: A Case Report.
CureusSirolimus is effective and safe in childhood relapsed-refractory autoimmune cytopenias: A multicentre study.
Scandinavian journal of immunologyEvans syndrome in the background of 22q11.2 deletion syndrome.
Pediatric blood & cancerSpectrum of renal disease in scleroderma other than scleroderma renal crisis: A review of the literature.
Clinical nephrologyParaneoplastic Syndromes in Neuroendocrine Prostate Cancer: A Systematic Review.
Current oncology (Toronto, Ont.)Daratumumab monotherapy in refractory warm autoimmune hemolytic anemia and cold agglutinin disease.
Blood advancesAutoimmune cytopenia and Kabuki syndrome in paediatrics: Insights in 11 patients.
British journal of haematologyPathological mutations reveal the key role of the cytosolic iRhom2 N-terminus for phosphorylation-independent 14-3-3 interaction and ADAM17 binding, stability, and activity.
Cellular and molecular life sciences : CMLSRecurrent aphthous stomatitis: a rare finding in Evans syndrome.
Clinical and experimental dermatologyCase report: Refractory Evans syndrome in two patients with spondyloenchondrodysplasia with immune dysregulation treated successfully with JAK1/JAK2 inhibition.
Frontiers in immunologyDiagnostic evaluation of paediatric autoimmune lymphoproliferative immunodeficiencies (ALPID): a prospective cohort study.
The Lancet. HaematologyA Case of Evans' Syndrome after Multiple Injuries and Septic Complications.
Journal of emergencies, trauma, and shockAntinuclear antibody-associated autoimmune cytopenia in childhood is a risk factor for systemic lupus erythematosus.
BloodAutoimmune Cytopenias Are Highly Associated with Inborn Errors of Immunity and They May Be the Initial Presentations in Cases without Severe Infections.
International archives of allergy and immunologyChallenges of providing biochemistry results in a patient with Evans syndrome.
Biochemia medicaSystemic lupus erythematosus mimicking retinal migraine: a case report.
Cephalalgia : an international journal of headacheAutoimmune lymphoproliferative immunodeficiencies (ALPIDs): A proposed approach to redefining ALPS and other lymphoproliferative immune disorders.
The Journal of allergy and clinical immunologySirolimus is effective for primary refractory/relapsed warm autoimmune haemolytic anaemia/Evans syndrome: a retrospective single-center study.
Annals of medicineThyroid and Adrenal Dysfunction in Hemoglobinopathies Before and After Allogeneic Hematopoietic Cell Transplant.
Journal of the Endocrine SocietyA pilot study of orelabrutinib treatment in three cases of refractory/relapsed autoimmune haemolytic anaemia/Evans syndrome.
British journal of haematologyMycophenolate mofetil for autoimmune cytopenias in children: high rates of response in inborn errors of immunity.
Frontiers in pediatricsEvaluating the prevalence of inborn errors of immunity in adults with chronic immune thrombocytopenia or Evans syndrome.
Blood advancesRobotic-Assisted Splenectomy by a Modified Lateral Approach: Technique and Outcomes.
CureusPaediatric-onset Evans syndrome: Breaking away from refractory immune thrombocytopenia.
British journal of haematologyT-large granular lymphocyte frequencies and correlates in disease states detected by multiparameter flow cytometry in pediatric and young adult population.
Annals of hematologyEvans syndrome during pembrolizumab therapy for upper urinary tract cancer.
IJU case reportsSusceptive piperacillin-tazobactam-induced Evans syndrome after radical resection of esophageal cancer: A rare case report.
Asian journal of surgeryEvans syndrome caused by a deleterious mutation affecting the adaptor protein SASH3.
British journal of haematologyClinical utility of measuring CD4+ T follicular cells in patients with immune dysregulation.
Journal of autoimmunityA woman with Sjogren's syndrome and a new diagnosis of Evans syndrome: a case report.
AME case reportsMassive Right Chylothorax Secondary to a Severe Systemic Lupus Erythematosus Flare With Secondary Evans Syndrome: A Case Report and Literature Review.
Clinical medicine insights. Case reportsBabesia gibsoni Infection in a Cat with Immune-Mediated Haemolytic Anaemia and Thrombocytopenia.
Animals : an open access journal from MDPICentral nervous system manifestations of LRBA deficiency: case report of two siblings and literature review.
BMC pediatricsAre accessory spleen screening and resection in refractory immune cytopenia an effective strategy or a waste of resources?
Annals of hematologyRelapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review.
Journal of infection in developing countriesImmune dysregulation in Kabuki syndrome: a case report of Evans syndrome and hypogammaglobulinemia.
Frontiers in pediatricsSystemic lupus erythematosus combined with Evans syndrome and primary biliary cirrhosis: A rare case.
Asian journal of surgeryAdvances in the diagnosis and treatment of Evans syndrome in children.
Asian journal of surgeryNeurological worsening during treatment of HIV-negative cryptococcal meningitis in a patient with Evans syndrome.
Future microbiologyMonogenic inborn errors of immunity in autoimmune disorders.
Immunologic researchAutoimmune Hemolytic Anemia in the Pediatric Age Group: The Egyptian Experience.
Annals of hematologyMaintenence rituximab following induction in autoimmune cytopenias.
British journal of haematologyOphthalmic Manifestations as First Presenting Sign of Evans Syndrome.
Journal of vitreoretinal diseasesA case of immune-mediated thrombocytopenia, possibly Evans syndrome, in a neutered Rottweiler dog.
The Canadian veterinary journal = La revue veterinaire canadienneEvans Syndrome as a Possible Complication of Brentuximab Vedotin Therapy for Peripheral T Cell Lymphoma.
Hematology reportsNaproxen-Induced Evans Syndrome.
CureusA case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura.
Modern rheumatology case reportsEvans' syndrome induced by atezolizumab plus bevacizumab combination therapy in advanced hepatocellular carcinoma.
Clinical journal of gastroenterology[Evans syndrome as initial presentation of COVID-19 infection: A case report and review of the literature].
Annales de biologie cliniqueDaratumumab as a novel treatment option in refractory ITP.
Blood cells, molecules & diseasesTherapeutic Options in Refractory Evans Syndrome: A Case Report.
CureusAutoimmune Hemolytic Anemia After Cord Blood Transplantation: A Retrospective Single-Center Experience.
Journal of inflammation researchUse of steroids in the management of low-risk myelodysplastic syndromes with autoimmune features.
Blood transfusion = Trasfusione del sangueRelapses are common in severe hematologic systemic lupus erythematosus and may be prevented by early institution of immunosuppressive agents: Α real-life single-center study.
LupusEvans' syndrome following vaccination with ChAdOx1 nCoV-19 in a patient with new-onset localized scleroderma.
Dermatology reportsAcute Isolated Thenar Compartment Syndrome in a Patient with Evans Syndrome: A Case Report.
Clinical practice and cases in emergency medicineCurrent clinical spectrum of common variable immunodeficiency in Spain: The multicentric nationwide GTEM-SEMI-CVID registry.
Frontiers in immunologyNew-onset Evans syndrome in a patient with SLE post SARS-CoV2 mRNA vaccination.
Annals of hematologyA Case of Secondary Hemophagocytic Lymphohistiocytosis Presenting with Severe Dyserythropoeisis in a Patient with Autoimmune Hemolysis.
Case reports in hematologyRefractory/relapse thrombocytopenia in a patient with Evans' syndrome successfully treated with zanubrutinib.
British journal of haematologyInfrequent Thrombotic Complications in Japanese Patients with Warm Autoimmune Hemolytic Anemia.
Internal medicine (Tokyo, Japan)Evans syndrome in a young man with rare autoimmune associations and transplanted liver.
BMJ case reportsEfficacy and steroid-sparing effect of tacrolimus in patients with autoimmune cytopenia.
Annals of hematologyUnexpected Thrombocytopenia in a Parturient With Evans Syndrome Complicated by COVID-19 Infection.
CureusComments on 'A rare haematological disorder in a Sudanese child: Evans syndrome, case report and literature review'.
Sudanese journal of paediatricsComplicated multiple organ infection of Purpureocillium lilacinum and varicella-zoster virus infection in a patient with Evans' syndrome.
Blood science (Baltimore, Md.)Commensalism or symbiosis: The potential use of rituximab in steroid-refractory Evans syndrome in a patient with ulcerative colitis.
Archives of rheumatologyEvans Syndrome Presenting as an Atypical Complication of SARS-CoV-2 Vaccination.
CureusAn event of Evans even in HIV.
Indian journal of sexually transmitted diseases and AIDSPrevalence and Characteristics of Cytomegalovirus Ocular Disease in Children: A Multi-Center Study.
Clinical ophthalmology (Auckland, N.Z.)Evans Syndrome in the Context of Incomplete Systemic Lupus Erythematosus.
CureusA Case Report of Autoimmune Phenomena With Underlying Breast Cancer: Occam's Razor Versus Hickam's Dictum.
CureusThrombopoietin receptor agonists in adult Evans syndrome: an international multicenter experience.
BloodCOVID-19 Vaccines and Autoimmune Hematologic Disorders.
VaccinesConcurrent severe pulmonary tuberculosis with Evans syndrome: a case report with literature review.
BMC infectious diseasesUnderlying Inborn Errors of Immunity in Patients With Evans Syndrome and Multilineage Cytopenias: A Single-Centre Analysis.
Frontiers in immunologyParaneoplastic Evans Syndrome in a Patient With Prostate Cancer With Small Cell Transformation.
CureusEvans Syndrome: A Case Report.
JNMA; journal of the Nepal Medical AssociationEfficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers.
Therapeutic advances in hematologySeroconversion to mRNA SARS-CoV-2 vaccines in patients with autoimmune cytopenias and bone marrow failures.
Scientific reportsCase Report: Use of Obinutuzumab as an Alternative Monoclonal Anti-CD20 Antibody in a Patient With Refractory Immune Thrombocytopenia Complicated by Rituximab-Induced Serum Sickness and Anti-Rituximab Antibodies.
Frontiers in immunologyPathogenesis of Autoimmune Cytopenias in Inborn Errors of Immunity Revealing Novel Therapeutic Targets.
Frontiers in immunologyDeterminants of long-term outcomes of splenectomy in pediatric autoimmune cytopenias.
BloodComplete recovery of deep venous thrombosis from Coombs (+) thrombotic thrombocytopenic purpura: case report.
Journal of cardiothoracic surgeryImpact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia.
Hematology, transfusion and cell therapyA rare case of calvarial tuberculosis in post-COVID-19 scenario.
International journal of mycobacteriologyPediatric Evans Syndrome: A 20-year experience from a tertiary center in Brazil.
Hematology, transfusion and cell therapyPrimary antiphospholipid syndrome in pediatrics: beyond thrombosis. Report of 32 cases and review of the evidence.
Pediatric rheumatology online journal[Refractory thrombotic thrombocytopenic purpura complicated with multiple cerebral infarction].
[Rinsho ketsueki] The Japanese journal of clinical hematologyRefractory immune thrombocytopenia successfully treated with bortezomib in a child with 22q11.2 deletion syndrome, complicated by Evans syndrome and hypogammaglobulinemia.
PlateletsAutoimmune Cytopenias and Dysregulated Immunophenotype Act as Warning Signs of Inborn Errors of Immunity: Results From a Prospective Study.
Frontiers in immunologyEvans syndrome: pathology and genomic hubris.
BloodEvans syndrome in the SARS-CoV-2 era: "springing up like mushrooms".
Blood transfusion = Trasfusione del sangueCOVID-19 and Immune-Mediated RBC Destruction.
American journal of clinical pathologyA full-term pregnant woman with secondary Evans syndrome caused by severe coronavirus disease 2019: a case report.
Journal of medical case reportsCOVID-19-associated Evans syndrome: A case report and review of the literature.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisPrimary Evans syndrome in an adult man.
BMJ case reportsMisdiagnosis of sitosterolemia in a patient as Evans syndrome and familial hypercholesterolemia.
Journal of clinical lipidology[Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia].
MedicinaSevere neonatal autoimmune thrombocytopenia secondary to maternal Evans syndrome.
BMJ case reportsAbatacept is useful in autoimmune cytopenia with immunopathologic manifestations caused by CTLA-4 defects.
BloodA Case of Evans Syndrome and Unstable Angina.
Journal of medical casesNew-onset Evans syndrome associated with systemic lupus erythematosus after BNT162b2 mRNA COVID-19 vaccination.
International journal of hematologyEvans syndrome in adults: an observational multicenter study.
Blood advancesAutoimmune Hemolytic Anaemias in Pregnancy: Experience in a Tertiary Care Hospital in South India.
Journal of obstetrics and gynaecology of IndiaAn Uncharacteristic Presentation of Evans Syndrome Following Treatment With Dupilumab.
CureusT-follicular helper cell expansion and chronic T-cell activation are characteristic immune anomalies in Evans syndrome.
BloodDengue Infection and Its Relationship with Evans Syndrome: A Pediatric Case.
Case reports in medicineRight heart thrombus-in-transit in a patient with Evans syndrome: A case report.
MedicineRisk factors of hospitalisation for thrombosis in adults with primary immune thrombocytopenia, including disease-specific treatments: a French nationwide cohort study.
British journal of haematology[Late onset primary antiphospholipid syndrome in the older adult].
Revista medica del Instituto Mexicano del Seguro SocialAcute haemolysis by cold antibody during SARS-CoV-2 infection in a patient with Evans syndrome: a case report and literature review.
Blood transfusion = Trasfusione del sangueSUBHYALOID HEMORRHAGE IN EVANS SYNDROME.
Retinal cases & brief reportsCase Report: Post-Partum Complications of NFκB1 Deficiency Underscore a Need to Better Understand Primary Immunodeficiency Management During Pregnancy.
Frontiers in pediatricsDiagnosis of Evans syndrome.
The Pan African medical journalEvans Syndrome in a Jehovah's Witness.
CureusInborn errors of IKAROS and AIOLOS.
Current opinion in immunologyEvans syndrome with auto-immune neutropenia.
International journal of laboratory hematologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Investigating Biomarkers for Inborn Errors of Immunity in a Prospective Study of Patients With Autoimmune Cytopenia.
- Pediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.
- Azathioprine or mycophenolate mofetil for pediatric autoimmune cytopenia: a propensity score-matched study.
- Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.
- A multicentre analysis of efficacy, safety and molecular response correlates of fostamatinib in warm autoimmune haemolytic anaemia and Evans syndrome.
- Alcohol-Induced Acute Pancreatitis Complicated by Severe Direct Antiglobulin Test (DAT)-Negative Hemolytic Anemia and Thrombocytopenia: A Suspected Evans Syndrome-Like Immune Cytopenia.
- Comparison of Evans syndrome and isolated autoimmune cytopenias in SLE: a retrospective study.
- Anti-CD19 chimeric antigen receptor T cells in Evans syndrome of systemic lupus erythematosus.
- Combined mesenchymal stem cells and telitacicept therapy for Evans syndrome refractory to various immunosuppressive: a case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1959(Orphanet)
- MONDO:0016030(MONDO)
- GARD:6389(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q3109873(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
