Research & Literature
Explore the leading researchers and institutions driving advances in this area, and dive into the full body of literature that informs this resource.
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Inserm
Paris, France
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico
Milan, Italy
Sorbonne Université
Paris, France
Hôpital Necker-Enfants Malades
Paris, France
Centre Hospitalier Universitaire de Nantes
Nantes, France
Centre National de la Recherche Scientifique
Paris, France
Hôpital Arnaud de Villeneuve
Montpellier, France
University of Milan
Milan, Italy
Assistance Publique – Hôpitaux de Paris
Paris, France
Hôpital Purpan
Toulouse, France
References
References (71)
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Naik R
Hematology/oncology clinics of North America 2015; (29(3)):445-53.
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Immune Hemolysis: Diagnosis and Treatment Recommendations.
Barcellini W
Seminars in hematology 2015; (52(4)):304-12.
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Evans Syndrome in Children: Long-Term Outcome in a Prospective French National Observational Cohort.
Aladjidi N, Fernandes H, Leblanc T, et al.
Frontiers in pediatrics 2015; (3()):79 doi:10.3389/fped.2015.00079.
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Bleeding complications in immune thrombocytopenia.
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Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia.
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Disease markers 2015; (2015()):635670 doi:10.1155/2015/635670.
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Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study.
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Pediatric blood & cancer 2016; (63(7)):1238-43 doi:10.1002/pbc.25976.
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American journal of hematology 2017; (92(4)):381-387 doi:10.1002/ajh.24665.
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Pediatric-onset Evans syndrome: Heterogeneous presentation and high frequency of monogenic disorders including LRBA and CTLA4 mutations.
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Clinical immunology (Orlando, Fla.) 2018; (188()):52-57 doi:10.1016/j.clim.2017.12.009.
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Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.
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Mediterranean journal of hematology and infectious diseases 2018; (10(1)):e2018018 doi:10.4084/MJHID.2018.018.
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A Comprehensive Diagnostic Algorithm for Direct Antiglobulin Test-Negative Autoimmune Hemolytic Anemia Reveals the Relative Ratio of Three Mechanisms in a Single Laboratory.
Kamesaki T, Kajii E
Acta haematologica 2018; (140(1)):10-17 doi:10.1159/000488753.
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Evans syndrome: clinical perspectives, biological insights and treatment modalities.
Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D
Journal of blood medicine 2018; (9()):171-184 doi:10.2147/JBM.S176144.
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Immune thrombocytopenia: improving quality of life and patient outcomes.
Trotter P, Hill QA
Patient related outcome measures 2018; (9()):369-384 doi:10.2147/PROM.S140932.
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Pediatric Evans syndrome is associated with a high frequency of potentially damaging variants in immune genes.
Hadjadj J, Aladjidi N, Fernandes H, et al.
Blood 2019; (134(1)):9-21 doi:10.1182/blood-2018-11-887141.
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A case series of pediatric patients with direct antiglobulin test negative autoimmune hemolytic anemia.
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Transfusion 2019; (59(8)):2528-2531 doi:10.1111/trf.15350.
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Evans syndrome in adults - incidence, prevalence, and survival in a nationwide cohort.
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American journal of hematology 2019; (94(10)):1081-1090 doi:10.1002/ajh.25574.
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Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single-center retrospective study.
Sulpizio ED, Raghunathan V, Shatzel JJ, et al.
European journal of haematology 2020; (104(1)):55-58 doi:10.1111/ejh.13336.
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Typical and Atypical Hemolytic Uremic Syndrome in the Critically Ill.
Manrique-Caballero CL, Peerapornratana S, Formeck C, et al.
Critical care clinics 2020; (36(2)):333-356 doi:10.1016/j.ccc.2019.11.004.
PMID: 32172817 - 19
Evans syndrome in children below 13 years of age - A nationwide population-based cohort study.
Mannering N, Hansen DL, Frederiksen H
PloS one 2020; (15(4)):e0231284 doi:10.1371/journal.pone.0231284.
PMID: 32271826 - 20
New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia.
Berentsen S
Frontiers in immunology 2020; (11()):590 doi:10.3389/fimmu.2020.00590.
PMID: 32318071 - 21
Immune thrombocytopenia (ITP) World Impact Survey (I-WISh): Impact of ITP on health-related quality of life.
Cooper N, Kruse A, Kruse C, et al.
American journal of hematology 2021; (96(2)):199-207 doi:10.1002/ajh.26036.
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Acute Kidney Injury Caused by Evans Syndrome with Systemic Lupus Erythematosus and Systemic Sclerosis.
Matsuoka N, Watanabe H, Kurooka N, et al.
Internal medicine (Tokyo, Japan) 2021; (60(7)):1055-1060 doi:10.2169/internalmedicine.5976-20.
PMID: 33116016 - 23
Treatment of immune-mediated cytopenias in patients with primary immunodeficiencies and immune regulatory disorders (PIRDs).
Seidel MG
Hematology. American Society of Hematology. Education Program 2020; (2020(1)):673-679 doi:10.1182/hematology.2020000153.
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Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma
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Turkish journal of haematology : official journal of Turkish Society of Haematology 2021; (38(2)):145-150 doi:10.4274/tjh.galenos.2020.2020.0618.
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Autoimmune Hemolytic Anemia in the Pediatric Setting.
Voulgaridou A, Kalfa TA
Journal of clinical medicine 2021; (10(2)) doi:10.3390/jcm10020216.
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Long term follow-up of pediatric-onset Evans syndrome: broad immunopathological manifestations and high treatment burden.
Pincez T, Fernandes H, Leblanc T, et al.
Haematologica 2022; (107(2)):457-466 doi:10.3324/haematol.2020.271106.
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Refractory autoimmune cytopenias in pediatric Evans syndrome with underlying systemic immune dysregulation.
Grimes AB, Kim TO, Kirk SE, et al.
European journal of haematology 2021; (106(6)):783-787 doi:10.1111/ejh.13600.
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Immune cytopenias as a continuum in inborn errors of immunity: An in-depth clinical and immunological exploration.
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Immunity, inflammation and disease 2021; (9(2)):583-594 doi:10.1002/iid3.420.
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A rare haematological disorder in a Sudanese child: Evans syndrome, case report and literature review.
Bashir BA, Othman SA, Malik AA
Sudanese journal of paediatrics 2021; (21(1)):89-94 doi:10.24911/SJP.106-1583095341.
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Primary Immunodeficiency in Children With Autoimmune Cytopenias: Retrospective 154-Patient Cohort.
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Frontiers in immunology 2021; (12()):649182 doi:10.3389/fimmu.2021.649182.
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It takes two to thrombosis: Hemolysis and complement.
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Blood reviews 2021; (50()):100834 doi:10.1016/j.blre.2021.100834.
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[Diagnosis and classification of direct antiglobulin test-negative autoimmune hemolytic anemia].
Kamesaki T
[Rinsho ketsueki] The Japanese journal of clinical hematology 2021; (62(5)):456-462 doi:10.11406/rinketsu.62.456.
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Qualitative study to support the content validity of the immune thrombocytopenia (ITP) Life Quality Index (ILQI).
Cooper N, Cuker A, Bonner N, et al.
British journal of haematology 2021; (194(4)):759-766 doi:10.1111/bjh.17694.
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Right heart thrombus-in-transit in a patient with Evans syndrome: A case report.
Yang YC, Chen YY
Medicine 2021; (100(33)):e27009 doi:10.1097/MD.0000000000027009.
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T-follicular helper cell expansion and chronic T-cell activation are characteristic immune anomalies in Evans syndrome.
Kumar D, Prince C, Bennett CM, et al.
Blood 2022; (139(3)):369-383 doi:10.1182/blood.2021012924.
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An Uncharacteristic Presentation of Evans Syndrome Following Treatment With Dupilumab.
Rutherford M, Tran M, Salazar L, et al.
Cureus 2021; (13(7)):e16658 doi:10.7759/cureus.16658.
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Evans syndrome in adults: an observational multicenter study.
Fattizzo B, Michel M, Giannotta JA, et al.
Blood advances 2021; (5(24)):5468-5478 doi:10.1182/bloodadvances.2021005610.
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New-onset Evans syndrome associated with systemic lupus erythematosus after BNT162b2 mRNA COVID-19 vaccination.
Hidaka D, Ogasawara R, Sugimura S, et al.
International journal of hematology 2022; (115(3)):424-427 doi:10.1007/s12185-021-03243-2.
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Assessment and Management of Immune Thrombocytopenia (ITP) in the Emergency Department: Current Perspectives.
Zitek T, Weber L, Pinzon D, Warren N
Open access emergency medicine : OAEM 2022; (14()):25-34 doi:10.2147/OAEM.S331675.
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Adult Evans' Syndrome.
Michel M
Hematology/oncology clinics of North America 2022; (36(2)):381-392 doi:10.1016/j.hoc.2021.12.004.
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DAT-Negative Autoimmune Hemolytic Anemia.
Rodberg K
Hematology/oncology clinics of North America 2022; (36(2)):307-313 doi:10.1016/j.hoc.2021.11.004.
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Determinants of long-term outcomes of splenectomy in pediatric autoimmune cytopenias.
Pincez T, Aladjidi N, Héritier S, et al.
Blood 2022; (140(3)):253-261 doi:10.1182/blood.2022015508.
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Severe immune thrombocytopenia following diphtheria, tetanus, pertussis and polio vaccination in a 36-year-old Caucasian woman: a case report.
Küster O, Schmohl J, Greiner J, Storz MA
European journal of medical research 2022; (27(1)):63 doi:10.1186/s40001-022-00686-z.
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Thrombotic thrombocytopenic purpura in pediatrics. A case report.
Rodas MA, Astorquizaga L, Lisanti C, et al.
Archivos argentinos de pediatria 2023; (121(5)):e202202758 doi:10.5546/aap.2022-02758.eng.
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The choice of new treatments in autoimmune hemolytic anemia: how to pick from the basket?
Berentsen S, Fattizzo B, Barcellini W
Frontiers in immunology 2023; (14()):1180509 doi:10.3389/fimmu.2023.1180509.
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Mixed Warm and Cold Autoimmune Hemolytic Anemia With Concomitant Immune Thrombocytopenia Following Recent SARS-CoV-2 Infection and Ongoing Rhinovirus Infection.
Karim F, Amardeep K, Yee A, et al.
Cureus 2023; (15(5)):e38509 doi:10.7759/cureus.38509.
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Hemoglobin cast nephropathy: a rare but serious complication of hemolysis in a pediatric patient.
Baltu D, Oral NA, Kesici S, et al.
The Turkish journal of pediatrics 2023; (65(5)):874-880.
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Antinuclear antibody-associated autoimmune cytopenia in childhood is a risk factor for systemic lupus erythematosus.
Granel J, Fernandes H, Bader-Meunier B, et al.
Blood 2024; (143(16)):1576-1585 doi:10.1182/blood.2023021884.
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Clinical and Treatment History of Patients with Partial DiGeorge Syndrome and Autoimmune Cytopenia at Multiple Centers.
Patel PK, Chinga ML, Yilmaz M, et al.
Journal of clinical immunology 2024; (44(2)):42 doi:10.1007/s10875-023-01607-3.
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Title not available
Hbibi M, El Alaoui El Hanafi M, Kasmi Z, et al.
La Tunisie medicale 2024; (102(1)):1-6 doi:10.62438/tunismed.v102i1.4503.
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Diagnosis and management of Evans syndrome in adults: first consensus recommendations.
Fattizzo B, Marchetti M, Michel M, et al.
The Lancet. Haematology 2024; (11(8)):e617-e628 doi:10.1016/S2352-3026(24)00144-3.
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Secondary Evans Syndrome Presenting With Lupus Anticoagulant.
Abdelhalim O, Serna C, Guerrero N, et al.
Cureus 2024; (16(7)):e63992 doi:10.7759/cureus.63992.
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[The treatment strategies of autoimmune hemolytic anemia].
Yue WR, Wu T, Wang XQ
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 2024; (45(6)):615-620 doi:10.3760/cma.j.cn121090-20231027-00236.
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Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.
Palvia AR, Damera AR, Magar S, et al.
Cureus 2024; (16(7)):e64866 doi:10.7759/cureus.64866.
PMID: 39156320 - 55
Autoimmune Hemolytic Anemias: Challenges in Diagnosis and Therapy.
Barcellini W, Fattizzo B
Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie 2024; (51(5)):321-331 doi:10.1159/000540475.
PMID: 39371250 - 56
A rare paraneoplastic condition in Hodgkin lymphoma: Evans syndrome and literature review.
Atas U, Cerci K, Tuncer S, Karakus V
Northern clinics of Istanbul 2024; (11(5)):488-491 doi:10.14744/nci.2022.66742.
PMID: 39431027 - 57
Consensus of the Italian Primary Immunodeficiency Network on the use and interpretation of genetic testing for diagnosing inborn errors of immunity.
Giardino G, Di Matteo G, Giliani S, et al.
The Journal of allergy and clinical immunology 2025; (155(4)):1149-1160 doi:10.1016/j.jaci.2024.11.030.
PMID: 39622296 - 58
Evans syndrome as a presentation in systemic lupus erythematous, coexisting with Hashimoto's thyroiditis and pernicious anemia: a case report.
Mansour M, Shamasnah A, Alsaadi D, et al.
Journal of medical case reports 2024; (18(1)):643 doi:10.1186/s13256-024-05002-3.
PMID: 39732732 - 59
Prolonged Severe CD4+ Lymphocytopenia and Hypogammaglobulinemia in Patients With Evans' Syndrome: A Case Report.
Kurokawa T, Imoto N, Muramatsu H, Shingo K
Cureus 2024; (16(12)):e75283 doi:10.7759/cureus.75283.
PMID: 39764316 - 60
Evans syndrome revisited.
Jiang D, Kuter DJ
Blood reviews 2025; (74()):101322 doi:10.1016/j.blre.2025.101322.
PMID: 40717001 - 61
Microangiopathic Anemia.
Bommer M, Bloehdorn J
Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie 2025; (52(4)):259-270 doi:10.1159/000544724.
PMID: 40809448 - 62
A Qualitative Study on Patient Experience with Signs, Symptoms, and Daily Impacts of Immune Thrombocytopenia.
Ghanima W, Cooper N, Bozzi S, et al.
The patient 2026; (19(1)):69-82 doi:10.1007/s40271-025-00762-6.
PMID: 40817963 - 63
Evans Syndrome: Analysis of 1255 Adult Cases in US Hospitals, Revealing Demographics, Associations, and Outcomes.
Patel R, Patel M, Laxmidhar F, et al.
International journal of hematology-oncology and stem cell research 2025; (19(2)):93-100 doi:10.18502/ijhoscr.v19i2.18546.
PMID: 40852700 - 64
Pediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.
Kim TO, Hillier K, Gunn E, et al.
Pediatric blood & cancer 2026; (73(4)):e70121 doi:10.1002/1545-5017.70121.
PMID: 41518117 - 65
Multifactorial thrombotic risk in Evans syndrome: a case report and review of the literature.
Erolmez HN, Eker S, Siddiqui F, et al.
Thrombosis journal 2026; (24(1)).
PMID: 41736014 - 66
Successful Treatment of Evans Syndrome Onset With Zanubrutinib in Combination With Romiplostim During Venetoclax Treatment in a Patient With Chronic Lymphocytic Leukemia: A Case Report.
Innao V, Barbagallo APM, Bianco O, et al.
Clinical case reports 2026; (14()):e72697 doi:10.1002/ccr3.72697.
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Warm Autoimmune Hemolytic Anemia in an Elderly Patient With a History of Chronic Idiopathic Thrombocytopenic Purpura: A Case Report.
Pitaro AT, Bhat S, Ali MZ, et al.
Cureus 2026; (18(5)):e108289 doi:10.7759/cureus.108289.
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Dissecting the direct antiglobulin test in warm autoimmune hemolytic anemia: clinical and laboratory correlations.
Blackall DP, Lewis SK
American journal of clinical pathology 2026; (165(6)) doi:10.1093/ajcp/aqag071.
PMID: 42287686 - 69
Unmasking Evans Syndrome: A Case of New Onset Anemia and Thrombocytopenia in a Pregnant Patient With Systemic Lupus Erythematosus.
Bittner C, Ryden NA, Jacocks C, Kou CJ
Case reports in hematology 2026; (2026()):9110168 doi:10.1155/crh/9110168.
PMID: 42396567 - 70
Cold agglutinin disease: pathophysiology, diagnosis, and the evolving therapeutic landscape.
Choi DH
Blood research 2026; (61(1)).
PMID: 42696222 - 71
Dual Autoimmunity: Evans Syndrome and Multiple Sclerosis.
Markl A, Warren M, Iskander J, et al.
Cureus 2026; (18(8)):e113997 doi:10.7759/cureus.113997.
PMID: 42698986