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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.
 
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

Professional guidelines

PubMed

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
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article
Norton A, Roberts I
Br J Haematol 2006 Jan;132(2):125-37. doi: 10.1111/j.1365-2141.2005.05809.x. PMID: 16398647

Recent clinical studies

Etiology

Hägele P, Staus P, Scheible R, Uhlmann A, Heeg M, Klemann C, Maccari ME, Ritterbusch H, Armstrong M, Cutcutache I, Elliott KS, von Bernuth H, Leahy TR, Leyh J, Holzinger D, Lehmberg K, Svec P, Masjosthusmann K, Hambleton S, Jakob M, Sparber-Sauer M, Kager L, Puzik A, Wolkewitz M, Lorenz MR, Schwarz K, Speckmann C, Rensing-Ehl A, Ehl S; ALPID study group
Lancet Haematol 2024 Feb;11(2):e114-e126. doi: 10.1016/S2352-3026(23)00362-9. PMID: 38302222
Torres-Jimenez AR, Ramirez-Nova V, Cespedes-Cruz AI, Sanchez-Jara B, Velazquez-Cruz A, Bekker-Méndez VC, Guerra-Castillo FX
Pediatr Rheumatol Online J 2022 Feb 14;20(1):13. doi: 10.1186/s12969-022-00673-y. PMID: 35164787Free PMC Article
Pincez T, Fernandes H, Leblanc T, Michel G, Barlogis V, Bertrand Y, Neven B, Chahla WA, Pasquet M, Guitton C, Marie-Cardine A, Pellier I, Armari-Alla C, Benadiba J, Blouin P, Jeziorski E, Millot F, Paillard C, Thomas C, Cheikh N, Bayart S, Fouyssac F, Piguet C, Deparis M, Briandet C, Dore E, Picard C, Rieux-Laucat F, Landman-Parker J, Leverger G, Aladjidi N
Haematologica 2022 Feb 1;107(2):457-466. doi: 10.3324/haematol.2020.271106. PMID: 33440924Free PMC Article
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
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C, Revelli N, Villa MA, Consonni D, Scaramucci L, De Fabritiis P, Tagariello G, Gaidano G, Rodeghiero F, Cortelezzi A, Zanella A
Blood 2014 Nov 6;124(19):2930-6. Epub 2014 Sep 16 doi: 10.1182/blood-2014-06-583021. PMID: 25232059

Diagnosis

Magerus A, Rensing-Ehl A, Rao VK, Teachey DT, Rieux-Laucat F, Ehl S
J Allergy Clin Immunol 2024 Jan;153(1):67-76. Epub 2023 Nov 17 doi: 10.1016/j.jaci.2023.11.004. PMID: 37977527Free PMC Article
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
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
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C, Revelli N, Villa MA, Consonni D, Scaramucci L, De Fabritiis P, Tagariello G, Gaidano G, Rodeghiero F, Cortelezzi A, Zanella A
Blood 2014 Nov 6;124(19):2930-6. Epub 2014 Sep 16 doi: 10.1182/blood-2014-06-583021. PMID: 25232059

Therapy

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
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
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
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C, Revelli N, Villa MA, Consonni D, Scaramucci L, De Fabritiis P, Tagariello G, Gaidano G, Rodeghiero F, Cortelezzi A, Zanella A
Blood 2014 Nov 6;124(19):2930-6. Epub 2014 Sep 16 doi: 10.1182/blood-2014-06-583021. PMID: 25232059
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

Prognosis

Pincez T, Fernandes H, Leblanc T, Michel G, Barlogis V, Bertrand Y, Neven B, Chahla WA, Pasquet M, Guitton C, Marie-Cardine A, Pellier I, Armari-Alla C, Benadiba J, Blouin P, Jeziorski E, Millot F, Paillard C, Thomas C, Cheikh N, Bayart S, Fouyssac F, Piguet C, Deparis M, Briandet C, Dore E, Picard C, Rieux-Laucat F, Landman-Parker J, Leverger G, Aladjidi N
Haematologica 2022 Feb 1;107(2):457-466. doi: 10.3324/haematol.2020.271106. PMID: 33440924Free PMC Article
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
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C, Revelli N, Villa MA, Consonni D, Scaramucci L, De Fabritiis P, Tagariello G, Gaidano G, Rodeghiero F, Cortelezzi A, Zanella A
Blood 2014 Nov 6;124(19):2930-6. Epub 2014 Sep 16 doi: 10.1182/blood-2014-06-583021. PMID: 25232059
Dave P, Krishna K, Diwan AG
J Assoc Physicians India 2012 Apr;60:60-1. PMID: 23029746

Clinical prediction guides

Cabañero-Navalon MD, Garcia-Bustos V, Nuñez-Beltran M, Císcar Fernández P, Mateu L, Solanich X, Carrillo-Linares JL, Robles-Marhuenda Á, Puchades-Gimeno F, Pelaez Ballesta A, López-Osle N, Torralba-Cabeza MÁ, Bielsa Masdeu AM, Diego Gil J, Tornador Gaya N, Pascual Castellanos G, Sánchez-Martínez R, Barragán-Casas JM, González-García A, Patier de la Peña JL, López-Wolf D, Mora Rufete A, Canovas Mora A, Forner Giner MJ, Moral Moral P
Front Immunol 2022;13:1033666. Epub 2022 Oct 28 doi: 10.3389/fimmu.2022.1033666. PMID: 36389743Free PMC Article
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
Barcellini W, Fattizzo B
Dis Markers 2015;2015:635670. Epub 2015 Dec 27 doi: 10.1155/2015/635670. PMID: 26819490Free PMC Article
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C, Revelli N, Villa MA, Consonni D, Scaramucci L, De Fabritiis P, Tagariello G, Gaidano G, Rodeghiero F, Cortelezzi A, Zanella A
Blood 2014 Nov 6;124(19):2930-6. Epub 2014 Sep 16 doi: 10.1182/blood-2014-06-583021. PMID: 25232059
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

Abufaraj M, Ramadan R, Alkhatib A
Curr Oncol 2024 Mar 21;31(3):1618-1632. doi: 10.3390/curroncol31030123. PMID: 38534956Free PMC Article
Jacobs JW, Booth GS
Am J Clin Pathol 2022 Jun 7;157(6):844-851. doi: 10.1093/ajcp/aqab210. PMID: 34919640Free PMC Article
Taherifard E, Taherifard E, Movahed H, Mousavi MR
Hematology 2021 Dec;26(1):225-239. doi: 10.1080/16078454.2021.1881225. PMID: 33594951
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
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

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