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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

Fattizzo B, Marchetti M, Michel M, Cantoni S, Frederiksen H, Giordano G, Glenthøj A, González-López TJ, Murakhovskaya I, Napolitano M, Mingot ME, Arguello M, Patriarca A, Raso S, Vianelli N, Barcellini W
Lancet Haematol 2024 Aug;11(8):e617-e628. Epub 2024 Jul 2 doi: 10.1016/S2352-3026(24)00144-3. PMID: 38968944
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

Recent clinical studies

Etiology

Fattizzo B, Carrai V, Crugnola M, Baldacci E, Bellini M, Bosi C, Buzzatti E, Caramazza D, Carli G, Carpenedo M, Clissa C, Danesin C, De Paolis MR, Giannotta JA, Innao V, Marchetti M, Markovic U, Morotti A, Napolitano M, Patriarca A, Pettine L, Poloni A, Rivolti E, Rossi E, Santeremo TM, Santoro C, Zannier ME, Zaja F, Cantoni S, Palandri F, De Stefano V
Eur J Haematol 2024 Oct;113(4):472-476. Epub 2024 Jun 21 doi: 10.1111/ejh.14256. PMID: 39031658
Jeanpierre M, Cognard J, Tusseau M, Riller Q, Bui LC, Berthelet J, Laurent A, Crickx E, Parlato M, Stolzenberg MC, Suarez F, Leverger G, Aladjidi N, Collardeau-Frachon S, Pietrement C, Malphettes M, Froissart A, Bole-Feysot C, Cagnard N, Rodrigues Lima F, Walzer T, Rieux-Laucat F, Belot A, Mathieu AL
J Exp Med 2024 Sep 2;221(9) Epub 2024 Jul 19 doi: 10.1084/jem.20232337. PMID: 39028869Free PMC Article
Jalink M, Jacobs CF, Khwaja J, Evers D, Bruggeman C, Fattizzo B, Michel M, Crickx E, Hill QA, Jaeger U, Kater AP, Mäkelburg ABU, Breedijk A, Te Boekhorst PAW, Hoeks MPA, de Haas M, D'Sa S, Vos JMI
Blood Adv 2024 Jun 11;8(11):2622-2634. doi: 10.1182/bloodadvances.2024012585. PMID: 38507742Free PMC Article
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
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

Fattizzo B, Marchetti M, Michel M, Cantoni S, Frederiksen H, Giordano G, Glenthøj A, González-López TJ, Murakhovskaya I, Napolitano M, Mingot ME, Arguello M, Patriarca A, Raso S, Vianelli N, Barcellini W
Lancet Haematol 2024 Aug;11(8):e617-e628. Epub 2024 Jul 2 doi: 10.1016/S2352-3026(24)00144-3. PMID: 38968944
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
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
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

Therapy

Fattizzo B, Marchetti M, Michel M, Cantoni S, Frederiksen H, Giordano G, Glenthøj A, González-López TJ, Murakhovskaya I, Napolitano M, Mingot ME, Arguello M, Patriarca A, Raso S, Vianelli N, Barcellini W
Lancet Haematol 2024 Aug;11(8):e617-e628. Epub 2024 Jul 2 doi: 10.1016/S2352-3026(24)00144-3. PMID: 38968944
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
Angelopoulos A, Kirkilesis G, Kakavia K, Papanikolaou P
Pan Afr Med J 2021;38:314. Epub 2021 Mar 29 doi: 10.11604/pamj.2021.38.314.22410. PMID: 34285737Free 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, 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

Prognosis

Fattizzo B, Marchetti M, Michel M, Cantoni S, Frederiksen H, Giordano G, Glenthøj A, González-López TJ, Murakhovskaya I, Napolitano M, Mingot ME, Arguello M, Patriarca A, Raso S, Vianelli N, Barcellini W
Lancet Haematol 2024 Aug;11(8):e617-e628. Epub 2024 Jul 2 doi: 10.1016/S2352-3026(24)00144-3. PMID: 38968944
Aladjidi N, Pincez T, Rieux-Laucat F, Nugent D
Br J Haematol 2023 Oct;203(1):28-35. doi: 10.1111/bjh.19073. PMID: 37735545
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
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

Clinical prediction guides

Jeanpierre M, Cognard J, Tusseau M, Riller Q, Bui LC, Berthelet J, Laurent A, Crickx E, Parlato M, Stolzenberg MC, Suarez F, Leverger G, Aladjidi N, Collardeau-Frachon S, Pietrement C, Malphettes M, Froissart A, Bole-Feysot C, Cagnard N, Rodrigues Lima F, Walzer T, Rieux-Laucat F, Belot A, Mathieu AL
J Exp Med 2024 Sep 2;221(9) Epub 2024 Jul 19 doi: 10.1084/jem.20232337. PMID: 39028869Free PMC Article
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
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

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
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
Letchumanan P, Thumboo J
Semin Arthritis Rheum 2011 Feb;40(4):298-306. Epub 2010 Jun 11 doi: 10.1016/j.semarthrit.2010.03.005. PMID: 20541792
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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