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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

Mantadakis E, Farmaki E
J Pediatr Hematol Oncol 2017 Aug;39(6):413-419. doi: 10.1097/MPH.0000000000000897. PMID: 28654461
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

Rai MP, Lee EJ, Bussel JB
Br J Haematol 2023 Jul;202(1):153-158. Epub 2023 Apr 22 doi: 10.1111/bjh.18814. PMID: 37086173
Hansen DL, Möller S, Andersen K, Gaist D, Frederiksen H
Am J Hematol 2019 Oct;94(10):1081-1090. Epub 2019 Aug 9 doi: 10.1002/ajh.25574. PMID: 31292991
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
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
Norton A, Roberts I
Br J Haematol 2006 Jan;132(2):125-37. doi: 10.1111/j.1365-2141.2005.05809.x. PMID: 16398647

Diagnosis

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
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
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

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
Mantadakis E, Farmaki E
J Pediatr Hematol Oncol 2017 Aug;39(6):413-419. doi: 10.1097/MPH.0000000000000897. PMID: 28654461
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
Miano M
Br J Haematol 2016 Feb;172(4):524-34. Epub 2015 Dec 2 doi: 10.1111/bjh.13866. PMID: 26625877
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

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
Pincez T, Neven B, Le Pointe HD, Varlet P, Fernandes H, Gareton A, Leverger G, Leblanc T, Chambost H, Michel G, Pasquet M, Millot F, Hermine O, Mathian A, Hully M, Zephir H, Hamidou M, Durand JM, Perel Y, Landman-Parker J, Rieux-Laucat F, Aladjidi N
J Clin Immunol 2019 Feb;39(2):171-181. Epub 2019 Jan 22 doi: 10.1007/s10875-019-0594-3. PMID: 30671780
Mantadakis E, Farmaki E
J Pediatr Hematol Oncol 2017 Aug;39(6):413-419. doi: 10.1097/MPH.0000000000000897. PMID: 28654461
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

Clinical prediction guides

Venkatachari IV, Chougule A, Gowri V, Taur P, Bodhanwala M, Prabhu S, Madkaikar M, Desai M
Immunol Res 2023 Oct;71(5):771-780. Epub 2023 May 18 doi: 10.1007/s12026-023-09391-3. PMID: 37199901
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
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
Costallat GL, Appenzeller S, Costallat LT
Joint Bone Spine 2012 Jul;79(4):362-4. Epub 2011 Sep 23 doi: 10.1016/j.jbspin.2011.07.004. PMID: 21944976

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
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
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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