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Síndrome Evans
ORPHA:1959CID-10 · D69.3CID-11 · 3A20.5DOENÇA RARA

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.

Mantido por Agente Raras·Colaborar como especialista →

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.

Pesquisas ativas
4 ensaios
15 total registrados no ClinicalTrials.gov
Publicações científicas
799 artigos
Último publicado: 2026 Mar

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.1
Europe
Início
Adult
+ childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D69.3
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
4 sintomas
💪
Músculos
1 sintomas
🫁
Pulmão
1 sintomas

+ 7 sintomas em outras categorias

Características mais comuns

100%prev.
Trombocitopenia autoimune
100%prev.
Anemia hemolítica autoimune
90%prev.
Petéquias
Muito frequente (99-80%)
90%prev.
Neutropenia na presença de anticorpos anti-neutrófilos
Muito frequente (99-80%)
55%prev.
Fraqueza muscular
Frequente (79-30%)
55%prev.
Epistaxe
Frequente (79-30%)
13sintomas
Muito frequente (4)
Frequente (8)
Ocasional (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.

Trombocitopenia autoimuneAutoimmune thrombocytopenia
Muito frequente100%
Anemia hemolítica autoimuneAutoimmune hemolytic anemia
Muito frequente100%
PetéquiasPetechiae
Muito frequente (99-80%)90%
Neutropenia na presença de anticorpos anti-neutrófilosNeutropenia in presence of anti-neutropil antibodies
Muito frequente (99-80%)90%
Fraqueza muscularMuscle weakness
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico799PubMed
Últimos 10 anos200publicações
Pico202245 papers
Linha do tempo
2026Hoje · 2026🧪 1997Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
2Fase 23
1Fase 11
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 8 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome Evans

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

15 ensaios clínicos encontrados, 4 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
396 papers (10 anos)
#1

Investigating Biomarkers for Inborn Errors of Immunity in a Prospective Study of Patients With Autoimmune Cytopenia.

Pediatric blood &amp; cancer2026 Mar

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.

#2

Pediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.

Pediatric blood &amp; cancer2026 Apr

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.

#3

Azathioprine or mycophenolate mofetil for pediatric autoimmune cytopenia: a propensity score-matched study.

Blood advances2026 Feb 10

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.

#4

Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.

Pediatric reports2026 Mar 03

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.

#5

A multicentre analysis of efficacy, safety and molecular response correlates of fostamatinib in warm autoimmune haemolytic anaemia and Evans syndrome.

British journal of haematology2026 Mar 17

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

Ver todas no PubMed

📚 EuropePMC516 artigos no totalmostrando 195

2026

Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.

Pediatric reports
2026

A multicentre analysis of efficacy, safety and molecular response correlates of fostamatinib in warm autoimmune haemolytic anaemia and Evans syndrome.

British journal of haematology
2026

Case Report: Native aortic valve Listeria monocytogenes endocarditis in an adult with Evans syndrome.

Frontiers in immunology
2026

Predictive factors for severity and poor treatment response in children with Evans syndrome: A retrospective cohort study.

Frontiers in immunology
2026

Circulating T follicular helper cells as emerging biomarkers in pediatric Evans syndrome.

Haematologica
2026

From Hemolysis to Lupus: A Case of Evans Syndrome Revealing Systemic Autoimmunity.

Clinical case reports
2026

Multifactorial thrombotic risk in Evans syndrome: a case report and review of the literature.

Thrombosis journal
2025

Multisystem Complications in Postpartum-Onset Evans Syndrome: A Case Report.

Cureus
2026

Immune-Mediated Multilineage Cytopenias in Thymoma: A Retrospective Case Series.

Thoracic cancer
2026

Investigating Biomarkers for Inborn Errors of Immunity in a Prospective Study of Patients With Autoimmune Cytopenia.

Pediatric blood &amp; cancer
2026

Pediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.

Pediatric blood &amp; cancer
2025

Incidence, Risk Factors, and Treatment of Autoimmune Cytopenia Following Pediatric Allogeneic Hematopoietic Stem Cell Transplantation.

Journal of transplantation
2026

Antiphospholipid Syndrome Coexisting With Evans Syndrome and SCL-70 Antibody Positivity: A Case Report.

Clinical case reports
2025

Double Trouble: The First Reported Case of Evans Syndrome Following RSV Vaccination.

Hematology reports
2025

What We Miss in Patients With Persistent Thrombocytopenia During the Dengue Season.

Cureus
2025

Severe relapse of Evans syndrome in an adult patient with treatment resistance and fatal outcome: A case report.

The Journal of international medical research
2025

Community-acquired Acinetobacter pneumonia associated with Evans syndrome: a case report.

Annals of medicine and surgery (2012)
2025

Exacerbation of Evans syndrome in vaccinated pregnant woman with mild COVID-19 infection.

AJOG global reports
2025

Successful Treatment of Evans Syndrome With efgartigimod: A Case Report.

American journal of therapeutics
2025

Hematological toxicity of immune checkpoint inhibitors: a pharmacovigilance study.

BMC cancer
2025

Transient Haemolytic Anaemia and Thrombocytopenia in a Healthy Young Adult Following Influenza a Infection.

European journal of case reports in internal medicine
2025

A New Variant in CTLA4 Highlights the Heterogeneous Phenotype of CTLA4 Haploinsufficiency.

Journal of clinical immunology
2026

Evidence 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 therapeutics
2025

Increased percentages of circulating T follicular helper cells associate with disease subtype and activity in pediatric immune cytopenias.

Haematologica
2026

Azathioprine or mycophenolate mofetil for pediatric autoimmune cytopenia: a propensity score-matched study.

Blood advances
2025

Evans Syndrome in a 35-Year-Old Male: Diagnostic Challenges and Multimodal Management.

Cureus
2025

Oxaliplatin-induced type II hypersensitivity in colorectal cancer: a cohort study on clinical presentation, diagnosis, and management.

Frontiers in pharmacology
2025

[Efficacy and Safety of Zanubrutinib in the Treatment of Autoimmune Cytopenia Secondary to Indolent B-Cell Lymphoma].

Zhongguo shi yan xue ye xue za zhi
2025

Evans syndrome in pregnancy and the postpartum period: A retrospective cohort study.

British journal of haematology
2025

Effectiveness 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 Immunology
2025

Acute Hemolytic Anemia and Immune Thrombocytopenia: A Rare Case of Parvovirus B19-Induced Evans Syndrome.

Cureus
2025

Severe 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)
2025

Evans Syndrome: Analysis of 1255 Adult Cases in US Hospitals, Revealing Demographics, Associations, and Outcomes.

International journal of hematology-oncology and stem cell research
2025

Autoimmune/Inflammatory Syndrome Induced by Adjuvants (ASIA) in Breast Implants: A Case Report.

Cureus
2025

Thrombotic storm with antiphospholipid syndrome in the setting of relapsing Evans syndrome and systemic lupus erythematosus: a case report.

AME case reports
2025

Evans syndrome revisited.

Blood reviews
2025

Primary antiphospholipid syndrome in pediatrics: New criteria, new opportunities.

Lupus
2025

Whole Exome Sequencing of Adult Patients With Evans Syndrome Reveals Pathogenic Variants Associated With Autoimmunity.

American journal of hematology
2025

Immunotherapy-associated hemostatic abnormalities: bleeding and thrombotic complications.

Annals of hematology
2025

Thrombotic Thrombocytopenic Purpura and Evans Syndrome: Validating and Exploring 20 Years of Routine Hospital Care.

Journal of blood medicine
2025

Evans Syndrome and COVID-19 Infection or Vaccination: A Systematic Review of Case Reports.

Journal of hematology
2025

A Room for Long-Lived Plasma Cell Contribution in Immune Cytopenias?

Cancers
2025

A Case of Systemic Lupus Erythematosus Complicated by Secondary Evans Syndrome.

Cureus
2025

Lipopolysaccharide-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 immunology
2025

Concurrent pulmonary TB and histoplasmosis in an HIV-negative patient with Evans Syndrome.

IJTLD open
2025

Rituximab as an Effective Treatment for New-onset Evans Syndrome and Systemic Lupus Erythematosus with Lupus Nephritis.

Internal medicine (Tokyo, Japan)
2025

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 Sinicae
2025

T and NK cell functionality in a patient harboring heterozygous novel BCL11B p.Asp632fsAla∗91 and STX11 p.R129P mutations.

Heliyon
2025

Combination of Evans syndrome and COVID-19: a systematic review of reported cases.

Blood transfusion = Trasfusione del sangue
2025

Mixed histiocytic sarcoma in a Bernese Mountain Dog.

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2024

SOCS1 deficiency-crossroads of autoimmunity and autoinflammation-two case reports.

Frontiers in pediatrics
2024

Budd-Chiari Syndrome as an Initial Manifestation of Incomplete Systemic Lupus Erythematosus.

European journal of case reports in internal medicine
2024

Prolonged Severe CD4+ Lymphocytopenia and Hypogammaglobulinemia in Patients With Evans' Syndrome: A Case Report.

Cureus
2025

Cerebral aspergillosis in a patient with chronic lymphocytic leukaemia complicated by Evans syndrome.

BMJ case reports
2024

Evans syndrome as a presentation in systemic lupus erythematous, coexisting with Hashimoto's thyroiditis and pernicious anemia: a case report.

Journal of medical case reports
2024

Pulmonary and Medullary Tuberculosis: An Uncommon Cause of Evans Syndrome in Adults.

Cureus
2024

Beyond FAScinating: advances in diagnosis and management of autoimmune lymphoproliferative syndrome and activated PI3 kinase δ syndrome.

Hematology. American Society of Hematology. Education Program
2024

A rare paraneoplastic condition in Hodgkin lymphoma: Evans syndrome and literature review.

Northern clinics of Istanbul
2024

[Chinese expert consensus on the diagnosis and treatment of Evans syndrome (2024)].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2024

Evans syndrome in conjunction with rheumatoid arthritis and SLE: A unique case of autoimmune intersection.

Radiology case reports
2024

Rapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.

Allergologie select
2024

Granulomatous Brain Involvement in Common Variable Immunodeficiency: A Case Report.

Cureus
2024

Differential diagnosis of Guillain-Barré syndrome: steroid-responsive radiculopathy in Evans syndrome.

Neurological research and practice
2024

Thrombotic thrombocytopenic purpura masquerading as Evans syndrome.

American journal of hematology
2024

Elective splenectomy for hematological diseases: a vanishing indication.

Surgical endoscopy
2024

Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.

Cureus
2024

Association of paediatric autoimmune cytopenia and inflammatory bowel disease suggests a common genetic origin.

British journal of haematology
2024

Secondary Evans Syndrome Presenting With Lupus Anticoagulant.

Cureus
2024

Familial autoimmunity and risk of developing immune thrombocytopenia and Evans syndrome.

Pediatric blood &amp; cancer
2024

Extremely Rare Case of Successful Treatment of Foot Ulcer Associated with Evans' Syndrome and Buerger's Disease.

Medicina (Kaunas, Lithuania)
2025

Navigating the conundrum of co-existing autoantibodies and alloantibodies in a case of Evans syndrome.

Laboratory medicine
2024

Evans syndrome: Disease awareness and clinical management in a nation-wide ITP-NET survey.

European journal of haematology
2024

Haploinsufficiency in PTPN2 leads to early-onset systemic autoimmunity from Evans syndrome to lupus.

The Journal of experimental medicine
2024

Evans syndrome suggests disease progression in lung adenocarcinoma.

Respiratory medicine case reports
2024

Recurrent Infection in a Young Female Patient Recently Diagnosed With Primary Evans Syndrome Without Neutropenia.

Journal of hematology
2024

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
2024

Diagnosis and management of Evans syndrome in adults: first consensus recommendations.

The Lancet. Haematology
2024

Spondyloenchondrodysplasia With Immune Dysregulation, but Without Skeletal Dysplasia, in a Six-Year-Old Boy: A Case Report.

Cureus
2024

Sirolimus is effective and safe in childhood relapsed-refractory autoimmune cytopenias: A multicentre study.

Scandinavian journal of immunology
2024

Evans syndrome in the background of 22q11.2 deletion syndrome.

Pediatric blood &amp; cancer
2024

Spectrum of renal disease in scleroderma other than scleroderma renal crisis: A review of the literature.

Clinical nephrology
2024

Paraneoplastic Syndromes in Neuroendocrine Prostate Cancer: A Systematic Review.

Current oncology (Toronto, Ont.)
2024

Daratumumab monotherapy in refractory warm autoimmune hemolytic anemia and cold agglutinin disease.

Blood advances
2024

Autoimmune cytopenia and Kabuki syndrome in paediatrics: Insights in 11 patients.

British journal of haematology
2024

Pathological 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 : CMLS
2024

Recurrent aphthous stomatitis: a rare finding in Evans syndrome.

Clinical and experimental dermatology
2023

Case report: Refractory Evans syndrome in two patients with spondyloenchondrodysplasia with immune dysregulation treated successfully with JAK1/JAK2 inhibition.

Frontiers in immunology
2024

Diagnostic evaluation of paediatric autoimmune lymphoproliferative immunodeficiencies (ALPID): a prospective cohort study.

The Lancet. Haematology
2023

A Case of Evans' Syndrome after Multiple Injuries and Septic Complications.

Journal of emergencies, trauma, and shock
2024

Antinuclear antibody-associated autoimmune cytopenia in childhood is a risk factor for systemic lupus erythematosus.

Blood
2024

Autoimmune 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 immunology
2024

Challenges of providing biochemistry results in a patient with Evans syndrome.

Biochemia medica
2023

Systemic lupus erythematosus mimicking retinal migraine: a case report.

Cephalalgia : an international journal of headache
2024

Autoimmune lymphoproliferative immunodeficiencies (ALPIDs): A proposed approach to redefining ALPS and other lymphoproliferative immune disorders.

The Journal of allergy and clinical immunology
2023

Sirolimus is effective for primary refractory/relapsed warm autoimmune haemolytic anaemia/Evans syndrome: a retrospective single-center study.

Annals of medicine
2023

Thyroid and Adrenal Dysfunction in Hemoglobinopathies Before and After Allogeneic Hematopoietic Cell Transplant.

Journal of the Endocrine Society
2024

A pilot study of orelabrutinib treatment in three cases of refractory/relapsed autoimmune haemolytic anaemia/Evans syndrome.

British journal of haematology
2023

Mycophenolate mofetil for autoimmune cytopenias in children: high rates of response in inborn errors of immunity.

Frontiers in pediatrics
2023

Evaluating the prevalence of inborn errors of immunity in adults with chronic immune thrombocytopenia or Evans syndrome.

Blood advances
2023

Robotic-Assisted Splenectomy by a Modified Lateral Approach: Technique and Outcomes.

Cureus
2023

Paediatric-onset Evans syndrome: Breaking away from refractory immune thrombocytopenia.

British journal of haematology
2024

T-large granular lymphocyte frequencies and correlates in disease states detected by multiparameter flow cytometry in pediatric and young adult population.

Annals of hematology
2023

Evans syndrome during pembrolizumab therapy for upper urinary tract cancer.

IJU case reports
2023

Susceptive piperacillin-tazobactam-induced Evans syndrome after radical resection of esophageal cancer: A rare case report.

Asian journal of surgery
2023

Evans syndrome caused by a deleterious mutation affecting the adaptor protein SASH3.

British journal of haematology
2023

Clinical utility of measuring CD4+ T follicular cells in patients with immune dysregulation.

Journal of autoimmunity
2023

A woman with Sjogren's syndrome and a new diagnosis of Evans syndrome: a case report.

AME case reports
2023

Massive Right Chylothorax Secondary to a Severe Systemic Lupus Erythematosus Flare With Secondary Evans Syndrome: A Case Report and Literature Review.

Clinical medicine insights. Case reports
2023

Babesia gibsoni Infection in a Cat with Immune-Mediated Haemolytic Anaemia and Thrombocytopenia.

Animals : an open access journal from MDPI
2023

Central nervous system manifestations of LRBA deficiency: case report of two siblings and literature review.

BMC pediatrics
2023

Are accessory spleen screening and resection in refractory immune cytopenia an effective strategy or a waste of resources?

Annals of hematology
2023

Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review.

Journal of infection in developing countries
2023

Immune dysregulation in Kabuki syndrome: a case report of Evans syndrome and hypogammaglobulinemia.

Frontiers in pediatrics
2023

Systemic lupus erythematosus combined with Evans syndrome and primary biliary cirrhosis: A rare case.

Asian journal of surgery
2023

Advances in the diagnosis and treatment of Evans syndrome in children.

Asian journal of surgery
2023

Neurological worsening during treatment of HIV-negative cryptococcal meningitis in a patient with Evans syndrome.

Future microbiology
2023

Monogenic inborn errors of immunity in autoimmune disorders.

Immunologic research
2023

Autoimmune Hemolytic Anemia in the Pediatric Age Group: The Egyptian Experience.

Annals of hematology
2023

Maintenence rituximab following induction in autoimmune cytopenias.

British journal of haematology
2022

Ophthalmic Manifestations as First Presenting Sign of Evans Syndrome.

Journal of vitreoretinal diseases
2023

A case of immune-mediated thrombocytopenia, possibly Evans syndrome, in a neutered Rottweiler dog.

The Canadian veterinary journal = La revue veterinaire canadienne
2023

Evans Syndrome as a Possible Complication of Brentuximab Vedotin Therapy for Peripheral T Cell Lymphoma.

Hematology reports
2023

Naproxen-Induced Evans Syndrome.

Cureus
2023

A case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura.

Modern rheumatology case reports
2023

Evans' syndrome induced by atezolizumab plus bevacizumab combination therapy in advanced hepatocellular carcinoma.

Clinical journal of gastroenterology
2023

[Evans syndrome as initial presentation of COVID-19 infection: A case report and review of the literature].

Annales de biologie clinique
2023

Daratumumab as a novel treatment option in refractory ITP.

Blood cells, molecules &amp; diseases
2022

Therapeutic Options in Refractory Evans Syndrome: A Case Report.

Cureus
2023

Autoimmune Hemolytic Anemia After Cord Blood Transplantation: A Retrospective Single-Center Experience.

Journal of inflammation research
2023

Use of steroids in the management of low-risk myelodysplastic syndromes with autoimmune features.

Blood transfusion = Trasfusione del sangue
2023

Relapses are common in severe hematologic systemic lupus erythematosus and may be prevented by early institution of immunosuppressive agents: Α real-life single-center study.

Lupus
2022

Evans' syndrome following vaccination with ChAdOx1 nCoV-19 in a patient with new-onset localized scleroderma.

Dermatology reports
2022

Acute Isolated Thenar Compartment Syndrome in a Patient with Evans Syndrome: A Case Report.

Clinical practice and cases in emergency medicine
2022

Current clinical spectrum of common variable immunodeficiency in Spain: The multicentric nationwide GTEM-SEMI-CVID registry.

Frontiers in immunology
2023

New-onset Evans syndrome in a patient with SLE post SARS-CoV2 mRNA vaccination.

Annals of hematology
2022

A Case of Secondary Hemophagocytic Lymphohistiocytosis Presenting with Severe Dyserythropoeisis in a Patient with Autoimmune Hemolysis.

Case reports in hematology
2022

Refractory/relapse thrombocytopenia in a patient with Evans' syndrome successfully treated with zanubrutinib.

British journal of haematology
2023

Infrequent Thrombotic Complications in Japanese Patients with Warm Autoimmune Hemolytic Anemia.

Internal medicine (Tokyo, Japan)
2022

Evans syndrome in a young man with rare autoimmune associations and transplanted liver.

BMJ case reports
2022

Efficacy and steroid-sparing effect of tacrolimus in patients with autoimmune cytopenia.

Annals of hematology
2022

Unexpected Thrombocytopenia in a Parturient With Evans Syndrome Complicated by COVID-19 Infection.

Cureus
2022

Comments on 'A rare haematological disorder in a Sudanese child: Evans syndrome, case report and literature review'.

Sudanese journal of paediatrics
2022

Complicated multiple organ infection of Purpureocillium lilacinum and varicella-zoster virus infection in a patient with Evans' syndrome.

Blood science (Baltimore, Md.)
2022

Commensalism or symbiosis: The potential use of rituximab in steroid-refractory Evans syndrome in a patient with ulcerative colitis.

Archives of rheumatology
2022

Evans Syndrome Presenting as an Atypical Complication of SARS-CoV-2 Vaccination.

Cureus
2022

An event of Evans even in HIV.

Indian journal of sexually transmitted diseases and AIDS
2022

Prevalence and Characteristics of Cytomegalovirus Ocular Disease in Children: A Multi-Center Study.

Clinical ophthalmology (Auckland, N.Z.)
2022

Evans Syndrome in the Context of Incomplete Systemic Lupus Erythematosus.

Cureus
2022

A Case Report of Autoimmune Phenomena With Underlying Breast Cancer: Occam's Razor Versus Hickam's Dictum.

Cureus
2022

Thrombopoietin receptor agonists in adult Evans syndrome: an international multicenter experience.

Blood
2022

COVID-19 Vaccines and Autoimmune Hematologic Disorders.

Vaccines
2022

Concurrent severe pulmonary tuberculosis with Evans syndrome: a case report with literature review.

BMC infectious diseases
2022

Underlying Inborn Errors of Immunity in Patients With Evans Syndrome and Multilineage Cytopenias: A Single-Centre Analysis.

Frontiers in immunology
2022

Paraneoplastic Evans Syndrome in a Patient With Prostate Cancer With Small Cell Transformation.

Cureus
2022

Evans Syndrome: A Case Report.

JNMA; journal of the Nepal Medical Association
2022

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers.

Therapeutic advances in hematology
2022

Seroconversion to mRNA SARS-CoV-2 vaccines in patients with autoimmune cytopenias and bone marrow failures.

Scientific reports
2022

Case 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 immunology
2022

Pathogenesis of Autoimmune Cytopenias in Inborn Errors of Immunity Revealing Novel Therapeutic Targets.

Frontiers in immunology
2022

Determinants of long-term outcomes of splenectomy in pediatric autoimmune cytopenias.

Blood
2022

Complete recovery of deep venous thrombosis from Coombs (+) thrombotic thrombocytopenic purpura: case report.

Journal of cardiothoracic surgery
2023

Impact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia.

Hematology, transfusion and cell therapy
2022

A rare case of calvarial tuberculosis in post-COVID-19 scenario.

International journal of mycobacteriology
2023

Pediatric Evans Syndrome: A 20-year experience from a tertiary center in Brazil.

Hematology, transfusion and cell therapy
2022

Primary antiphospholipid syndrome in pediatrics: beyond thrombosis. Report of 32 cases and review of the evidence.

Pediatric rheumatology online journal
2022

[Refractory thrombotic thrombocytopenic purpura complicated with multiple cerebral infarction].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2022

Refractory immune thrombocytopenia successfully treated with bortezomib in a child with 22q11.2 deletion syndrome, complicated by Evans syndrome and hypogammaglobulinemia.

Platelets
2021

Autoimmune Cytopenias and Dysregulated Immunophenotype Act as Warning Signs of Inborn Errors of Immunity: Results From a Prospective Study.

Frontiers in immunology
2022

Evans syndrome: pathology and genomic hubris.

Blood
2022

Evans syndrome in the SARS-CoV-2 era: "springing up like mushrooms".

Blood transfusion = Trasfusione del sangue
2022

COVID-19 and Immune-Mediated RBC Destruction.

American journal of clinical pathology
2021

A full-term pregnant woman with secondary Evans syndrome caused by severe coronavirus disease 2019: a case report.

Journal of medical case reports
2022

COVID-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 Haemapheresis
2021

Primary Evans syndrome in an adult man.

BMJ case reports
2022

Misdiagnosis of sitosterolemia in a patient as Evans syndrome and familial hypercholesterolemia.

Journal of clinical lipidology
2021

[Evans syndrome and rheumatoid arthritis as autoimmune manifestations of large granular T-cell leukemia].

Medicina
2021

Severe neonatal autoimmune thrombocytopenia secondary to maternal Evans syndrome.

BMJ case reports
2022

Abatacept is useful in autoimmune cytopenia with immunopathologic manifestations caused by CTLA-4 defects.

Blood
2021

A Case of Evans Syndrome and Unstable Angina.

Journal of medical cases
2022

New-onset Evans syndrome associated with systemic lupus erythematosus after BNT162b2 mRNA COVID-19 vaccination.

International journal of hematology
2021

Evans syndrome in adults: an observational multicenter study.

Blood advances
2021

Autoimmune Hemolytic Anaemias in Pregnancy: Experience in a Tertiary Care Hospital in South India.

Journal of obstetrics and gynaecology of India
2021

An Uncharacteristic Presentation of Evans Syndrome Following Treatment With Dupilumab.

Cureus
2022

T-follicular helper cell expansion and chronic T-cell activation are characteristic immune anomalies in Evans syndrome.

Blood
2021

Dengue Infection and Its Relationship with Evans Syndrome: A Pediatric Case.

Case reports in medicine
2021

Right heart thrombus-in-transit in a patient with Evans syndrome: A case report.

Medicine
2021

Risk factors of hospitalisation for thrombosis in adults with primary immune thrombocytopenia, including disease-specific treatments: a French nationwide cohort study.

British journal of haematology
2021

[Late onset primary antiphospholipid syndrome in the older adult].

Revista medica del Instituto Mexicano del Seguro Social
2022

Acute 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 sangue
2023

SUBHYALOID HEMORRHAGE IN EVANS SYNDROME.

Retinal cases &amp; brief reports
2021

Case Report: Post-Partum Complications of NFκB1 Deficiency Underscore a Need to Better Understand Primary Immunodeficiency Management During Pregnancy.

Frontiers in pediatrics
2021

Diagnosis of Evans syndrome.

The Pan African medical journal
2021

Evans Syndrome in a Jehovah's Witness.

Cureus
2021

Inborn errors of IKAROS and AIOLOS.

Current opinion in immunology
2022

Evans syndrome with auto-immune neutropenia.

International journal of laboratory hematology
Ver todos os 516 no EuropePMC

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Doenças relacionadas

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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.

  1. Investigating Biomarkers for Inborn Errors of Immunity in a Prospective Study of Patients With Autoimmune Cytopenia.
    Pediatric blood &amp; cancer· 2026· PMID 41560547mais citado
  2. Pediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.
    Pediatric blood &amp; cancer· 2026· PMID 41518117mais citado
  3. Azathioprine or mycophenolate mofetil for pediatric autoimmune cytopenia: a propensity score-matched study.
    Blood advances· 2026· PMID 41092477mais citado
  4. Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.
    Pediatric reports· 2026· PMID 41874013mais citado
  5. A multicentre analysis of efficacy, safety and molecular response correlates of fostamatinib in warm autoimmune haemolytic anaemia and Evans syndrome.
    British journal of haematology· 2026· PMID 41846299mais citado
  6. Alcohol-Induced Acute Pancreatitis Complicated by Severe Direct Antiglobulin Test (DAT)-Negative Hemolytic Anemia and Thrombocytopenia: A Suspected Evans Syndrome-Like Immune Cytopenia.
    Cureus· 2026· PMID 41970138recente
  7. Comparison of Evans syndrome and isolated autoimmune cytopenias in SLE: a retrospective study.
    Lupus Sci Med· 2026· PMID 41922007recente
  8. Anti-CD19 chimeric antigen receptor T cells in Evans syndrome of systemic lupus erythematosus.
    Ann Hematol· 2026· PMID 41920311recente
  9. Combined mesenchymal stem cells and telitacicept therapy for Evans syndrome refractory to various immunosuppressive: a case report.
    Res Pract Thromb Haemost· 2026· PMID 41890555recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1959(Orphanet)
  2. MONDO:0016030(MONDO)
  3. GARD:6389(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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

Síndrome Evans
Compêndio · Raras BR

Síndrome Evans

ORPHA:1959 · MONDO:0016030
Prevalência
1-9 / 1 000 000
Herança
Not applicable
CID-10
D69.3 · Púrpura trombocitopênica idiopática
CID-11
Ensaios
4 ativos
Início
Adult, Childhood
Prevalência
0.1 (Europe)
MedGen
UMLS
C0272126
EuropePMC
Wikidata
Papers 10a
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