U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Evans syndrome

MedGen UID:
75773
•Concept ID:
C0272126
•
Disease or Syndrome
Synonyms: Autoimmune hemolytic anemia and autoimmune thrombocytopenia; Evan syndrome
SNOMED CT: Evans syndrome (75331009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016030
Orphanet: ORPHA1959

Definition

A rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Evans syndrome in Orphanet.

Professional guidelines

PubMed

Primary and Secondary Immune Cytopenias: Evaluation and Treatment Approach in Children.
Kim TO, Despotovic JM
Hematol Oncol Clin North Am 2019 Jun;33(3):489-506. Epub 2019 Mar 23 doi: 10.1016/j.hoc.2019.01.005. PMID: 31030815
Guidelines on the management of drug-induced immune and secondary autoimmune, haemolytic anaemia.
Hill QA, Stamps R, Massey E, Grainger JD, Provan D, Hill A; British Society for Haematology Guidelines
Br J Haematol 2017 Apr;177(2):208-220. Epub 2017 Apr 3 doi: 10.1111/bjh.14654. PMID: 28369704
Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia.
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article

Recent clinical studies

Etiology

Autoimmune Cytopenias and Dysregulated Immunophenotype Act as Warning Signs of Inborn Errors of Immunity: Results From a Prospective Study.
Schiavo E, Martini B, Attardi E, Consonni F, Ciullini Mannurita S, Coniglio ML, Tellini M, Chiocca E, Fotzi I, Luti L, D'Alba I, Veltroni M, Favre C, Gambineri E
Front Immunol 2021;12:790455. Epub 2022 Jan 4 doi: 10.3389/fimmu.2021.790455. PMID: 35058929Free PMC Article
Evans syndrome in adults: an observational multicenter study.
Fattizzo B, Michel M, Giannotta JA, Hansen DL, Arguello M, Sutto E, Bianchetti N, Patriarca A, Cantoni S, Mingot-Castellano ME, McDonald V, Capecchi M, Zaninoni A, Consonni D, Vos JM, Vianelli N, Chen F, Glenthøj A, Frederiksen H, González-López TJ, Barcellini W
Blood Adv 2021 Dec 28;5(24):5468-5478. doi: 10.1182/bloodadvances.2021005610. PMID: 34592758Free PMC Article
Primary and Secondary Immune Cytopenias: Evaluation and Treatment Approach in Children.
Kim TO, Despotovic JM
Hematol Oncol Clin North Am 2019 Jun;33(3):489-506. Epub 2019 Mar 23 doi: 10.1016/j.hoc.2019.01.005. PMID: 31030815
Diagnosis and management of chronic and refractory immune cytopenias in children, adolescents, and young adults.
Rotz SJ, Ware RE, Kumar A
Pediatr Blood Cancer 2018 Oct;65(10):e27260. Epub 2018 Jun 1 doi: 10.1002/pbc.27260. PMID: 29856527
Treatment of refractory ITP and Evans syndrome by haematopoietic cell transplantation: is it indicated, and for whom?
Vaughn JE, Anwer F, Deeg HJ
Vox Sang 2016 Jan;110(1):5-11. Epub 2015 Jul 14 doi: 10.1111/vox.12314. PMID: 26178735Free PMC Article

Diagnosis

Adult Evans' Syndrome.
Michel M
Hematol Oncol Clin North Am 2022 Apr;36(2):381-392. Epub 2022 Mar 11 doi: 10.1016/j.hoc.2021.12.004. PMID: 35282950
COVID-19 and Immune-Mediated RBC Destruction.
Jacobs JW, Booth GS
Am J Clin Pathol 2022 Jun 7;157(6):844-851. doi: 10.1093/ajcp/aqab210. PMID: 34919640Free PMC Article
The diagnosis and management of the haematologic manifestations of lupus.
Velo-García A, Castro SG, Isenberg DA
J Autoimmun 2016 Nov;74:139-160. Epub 2016 Jul 25 doi: 10.1016/j.jaut.2016.07.001. PMID: 27461045
Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia.
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article
Pathobiology of secondary immune thrombocytopenia.
Cines DB, Liebman H, Stasi R
Semin Hematol 2009 Jan;46(1 Suppl 2):S2-14. doi: 10.1053/j.seminhematol.2008.12.005. PMID: 19245930Free PMC Article

Therapy

Autoimmune Cytopenias and Dysregulated Immunophenotype Act as Warning Signs of Inborn Errors of Immunity: Results From a Prospective Study.
Schiavo E, Martini B, Attardi E, Consonni F, Ciullini Mannurita S, Coniglio ML, Tellini M, Chiocca E, Fotzi I, Luti L, D'Alba I, Veltroni M, Favre C, Gambineri E
Front Immunol 2021;12:790455. Epub 2022 Jan 4 doi: 10.3389/fimmu.2021.790455. PMID: 35058929Free PMC Article
Disseminated Tuberculosis Associated Hemophagocytic Lymphohistiocytosis in a Pregnant Woman With Evans syndrome: A Case Report and Literature Review.
Shi YF, Shi XH, Zhang Y, Chen JX, Lai WX, Luo JM, Ba JH, Wang YH, Chen JN, Wu BQ
Front Immunol 2021;12:676132. Epub 2021 Jun 10 doi: 10.3389/fimmu.2021.676132. PMID: 34177917Free PMC Article
Primary and Secondary Immune Cytopenias: Evaluation and Treatment Approach in Children.
Kim TO, Despotovic JM
Hematol Oncol Clin North Am 2019 Jun;33(3):489-506. Epub 2019 Mar 23 doi: 10.1016/j.hoc.2019.01.005. PMID: 31030815
The diagnosis and management of the haematologic manifestations of lupus.
Velo-García A, Castro SG, Isenberg DA
J Autoimmun 2016 Nov;74:139-160. Epub 2016 Jul 25 doi: 10.1016/j.jaut.2016.07.001. PMID: 27461045
Treatment of refractory ITP and Evans syndrome by haematopoietic cell transplantation: is it indicated, and for whom?
Vaughn JE, Anwer F, Deeg HJ
Vox Sang 2016 Jan;110(1):5-11. Epub 2015 Jul 14 doi: 10.1111/vox.12314. PMID: 26178735Free PMC Article

Prognosis

Daratumumab as a novel treatment option in refractory ITP.
Vernava I, Schmitt CA
Blood Cells Mol Dis 2023 Mar;99:102724. Epub 2023 Jan 13 doi: 10.1016/j.bcmd.2023.102724. PMID: 36669360
Pathogenesis of Autoimmune Cytopenias in Inborn Errors of Immunity Revealing Novel Therapeutic Targets.
Cortesi M, Soresina A, Dotta L, Gorio C, Cattalini M, Lougaris V, Porta F, Badolato R
Front Immunol 2022;13:846660. Epub 2022 Apr 6 doi: 10.3389/fimmu.2022.846660. PMID: 35464467Free PMC Article
Evans syndrome in adults: an observational multicenter study.
Fattizzo B, Michel M, Giannotta JA, Hansen DL, Arguello M, Sutto E, Bianchetti N, Patriarca A, Cantoni S, Mingot-Castellano ME, McDonald V, Capecchi M, Zaninoni A, Consonni D, Vos JM, Vianelli N, Chen F, Glenthøj A, Frederiksen H, González-López TJ, Barcellini W
Blood Adv 2021 Dec 28;5(24):5468-5478. doi: 10.1182/bloodadvances.2021005610. PMID: 34592758Free PMC Article
Natural History, Pathogenesis, and Treatment of Evans Syndrome in Children.
Mantadakis E, Farmaki E
J Pediatr Hematol Oncol 2017 Aug;39(6):413-419. doi: 10.1097/MPH.0000000000000897. PMID: 28654461
Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia.
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article

Clinical prediction guides

Evans syndrome in adults: an observational multicenter study.
Fattizzo B, Michel M, Giannotta JA, Hansen DL, Arguello M, Sutto E, Bianchetti N, Patriarca A, Cantoni S, Mingot-Castellano ME, McDonald V, Capecchi M, Zaninoni A, Consonni D, Vos JM, Vianelli N, Chen F, Glenthøj A, Frederiksen H, González-López TJ, Barcellini W
Blood Adv 2021 Dec 28;5(24):5468-5478. doi: 10.1182/bloodadvances.2021005610. PMID: 34592758Free PMC Article
Clinical Aspects of STAT3 Gain-of-Function Germline Mutations: A Systematic Review.
Fabre A, Marchal S, Barlogis V, Mari B, Barbry P, Rohrlich PS, Forbes LR, Vogel TP, Giovannini-Chami L
J Allergy Clin Immunol Pract 2019 Jul-Aug;7(6):1958-1969.e9. Epub 2019 Feb 27 doi: 10.1016/j.jaip.2019.02.018. PMID: 30825606
The diagnosis and management of the haematologic manifestations of lupus.
Velo-García A, Castro SG, Isenberg DA
J Autoimmun 2016 Nov;74:139-160. Epub 2016 Jul 25 doi: 10.1016/j.jaut.2016.07.001. PMID: 27461045
Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia.
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article
Pathobiology of secondary immune thrombocytopenia.
Cines DB, Liebman H, Stasi R
Semin Hematol 2009 Jan;46(1 Suppl 2):S2-14. doi: 10.1053/j.seminhematol.2008.12.005. PMID: 19245930Free PMC Article

Recent systematic reviews

COVID-19 and Immune-Mediated RBC Destruction.
Jacobs JW, Booth GS
Am J Clin Pathol 2022 Jun 7;157(6):844-851. doi: 10.1093/ajcp/aqab210. PMID: 34919640Free PMC Article
Hematologic autoimmune disorders in the course of COVID-19: a systematic review of reported cases.
Taherifard E, Taherifard E, Movahed H, Mousavi MR
Hematology 2021 Dec;26(1):225-239. doi: 10.1080/16078454.2021.1881225. PMID: 33594951
Clinical Aspects of STAT3 Gain-of-Function Germline Mutations: A Systematic Review.
Fabre A, Marchal S, Barlogis V, Mari B, Barbry P, Rohrlich PS, Forbes LR, Vogel TP, Giovannini-Chami L
J Allergy Clin Immunol Pract 2019 Jul-Aug;7(6):1958-1969.e9. Epub 2019 Feb 27 doi: 10.1016/j.jaip.2019.02.018. PMID: 30825606
Rituximab for children with immune thrombocytopenia: a systematic review.
Liang Y, Zhang L, Gao J, Hu D, Ai Y
PLoS One 2012;7(5):e36698. Epub 2012 May 30 doi: 10.1371/journal.pone.0036698. PMID: 22666325Free PMC Article
Evans' syndrome in pregnancy: a systematic literature review and two new cases.
Lefkou E, Nelson-Piercy C, Hunt BJ
Eur J Obstet Gynecol Reprod Biol 2010 Mar;149(1):10-7. Epub 2009 Dec 23 doi: 10.1016/j.ejogrb.2009.11.022. PMID: 20031296

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...