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Hypereosinophilic syndrome

MedGen UID:
280990
•Concept ID:
C1540912
•
Disease or Syndrome
Synonyms: Hypereosinophilic Syndrome; Hypereosinophilic Syndromes; Syndrome, Hypereosinophilic; Syndromes, Hypereosinophilic
SNOMED CT: Hypereosinophilic syndrome (393573009); HES - hypereosinophilic syndrome (393573009); Hypereosinophilic syndrome (1156250009)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Unknown inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015691
Orphanet: ORPHA168956

Definition

Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage. [from ORDO]

Professional guidelines

PubMed

Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes.
Valent P, Klion AD, Roufosse F, Simon D, Metzgeroth G, Leiferman KM, Schwaab J, Butterfield JH, Sperr WR, Sotlar K, Vandenberghe P, Hoermann G, Haferlach T, Moriggl R, George TI, Akin C, Bochner BS, Gotlib J, Reiter A, Horny HP, Arock M, Simon HU, Gleich GJ
Allergy 2023 Jan;78(1):47-59. Epub 2022 Oct 19 doi: 10.1111/all.15544. PMID: 36207764Free PMC Article
Clinical Profile and Treatment in Hypereosinophilic Syndrome Variants: A Pragmatic Review.
Requena G, van den Bosch J, Akuthota P, Kovalszki A, Steinfeld J, Kwon N, Van Dyke MK
J Allergy Clin Immunol Pract 2022 Aug;10(8):2125-2134. Epub 2022 Apr 22 doi: 10.1016/j.jaip.2022.03.034. PMID: 35470096
Diagnosis and Management of Pediatric Hypereosinophilic Syndrome.
Rosenberg CE, Fulkerson PC, Williams KW
J Allergy Clin Immunol Pract 2022 May;10(5):1131-1138. Epub 2022 Feb 15 doi: 10.1016/j.jaip.2022.02.007. PMID: 35181546

Recent clinical studies

Etiology

Approach to the patient with suspected hypereosinophilic syndrome.
Klion AD
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):47-54. doi: 10.1182/hematology.2022000367. PMID: 36485140Free PMC Article
Clinical Profile and Treatment in Hypereosinophilic Syndrome Variants: A Pragmatic Review.
Requena G, van den Bosch J, Akuthota P, Kovalszki A, Steinfeld J, Kwon N, Van Dyke MK
J Allergy Clin Immunol Pract 2022 Aug;10(8):2125-2134. Epub 2022 Apr 22 doi: 10.1016/j.jaip.2022.03.034. PMID: 35470096
Diagnosis and Management of Pediatric Hypereosinophilic Syndrome.
Rosenberg CE, Fulkerson PC, Williams KW
J Allergy Clin Immunol Pract 2022 May;10(5):1131-1138. Epub 2022 Feb 15 doi: 10.1016/j.jaip.2022.02.007. PMID: 35181546
Eosinophilia.
Kovalszki A, Weller PF
Prim Care 2016 Dec;43(4):607-617. Epub 2016 Oct 14 doi: 10.1016/j.pop.2016.07.010. PMID: 27866580Free PMC Article
Hypereosinophilic Syndrome.
Curtis C, Ogbogu P
Clin Rev Allergy Immunol 2016 Apr;50(2):240-51. doi: 10.1007/s12016-015-8506-7. PMID: 26475367

Diagnosis

Approach to the patient with suspected hypereosinophilic syndrome.
Klion AD
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):47-54. doi: 10.1182/hematology.2022000367. PMID: 36485140Free PMC Article
Clinical Profile and Treatment in Hypereosinophilic Syndrome Variants: A Pragmatic Review.
Requena G, van den Bosch J, Akuthota P, Kovalszki A, Steinfeld J, Kwon N, Van Dyke MK
J Allergy Clin Immunol Pract 2022 Aug;10(8):2125-2134. Epub 2022 Apr 22 doi: 10.1016/j.jaip.2022.03.034. PMID: 35470096
Diagnosis and Management of Pediatric Hypereosinophilic Syndrome.
Rosenberg CE, Fulkerson PC, Williams KW
J Allergy Clin Immunol Pract 2022 May;10(5):1131-1138. Epub 2022 Feb 15 doi: 10.1016/j.jaip.2022.02.007. PMID: 35181546
The Diagnostic Work-Up of Hypereosinophilia.
Wang SA
Pathobiology 2019;86(1):39-52. Epub 2018 Jun 29 doi: 10.1159/000489341. PMID: 29961056
Hypereosinophilic Syndrome.
Curtis C, Ogbogu P
Clin Rev Allergy Immunol 2016 Apr;50(2):240-51. doi: 10.1007/s12016-015-8506-7. PMID: 26475367

Therapy

Biologics and Hypereosinophilic Syndromes: Knowledge Gaps and Controversies.
Kuang FL, Khoury P, Weller PF, Wechsler ME, Klion AD
J Allergy Clin Immunol Pract 2023 Sep;11(9):2666-2671. Epub 2023 Jul 26 doi: 10.1016/j.jaip.2023.07.026. PMID: 37507068Free PMC Article
Approach to the patient with suspected hypereosinophilic syndrome.
Klion AD
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):47-54. doi: 10.1182/hematology.2022000367. PMID: 36485140Free PMC Article
Clinical Profile and Treatment in Hypereosinophilic Syndrome Variants: A Pragmatic Review.
Requena G, van den Bosch J, Akuthota P, Kovalszki A, Steinfeld J, Kwon N, Van Dyke MK
J Allergy Clin Immunol Pract 2022 Aug;10(8):2125-2134. Epub 2022 Apr 22 doi: 10.1016/j.jaip.2022.03.034. PMID: 35470096
Efficacy and safety of mepolizumab in hypereosinophilic syndrome: A phase III, randomized, placebo-controlled trial.
Roufosse F, Kahn JE, Rothenberg ME, Wardlaw AJ, Klion AD, Kirby SY, Gilson MJ, Bentley JH, Bradford ES, Yancey SW, Steinfeld J, Gleich GJ; HES Mepolizumab study group
J Allergy Clin Immunol 2020 Dec;146(6):1397-1405. Epub 2020 Sep 18 doi: 10.1016/j.jaci.2020.08.037. PMID: 32956756Free PMC Article
Hypereosinophilic syndrome: approach to treatment in the era of precision medicine.
Klion A
Hematology Am Soc Hematol Educ Program 2018 Nov 30;2018(1):326-331. doi: 10.1182/asheducation-2018.1.326. PMID: 30504328Free PMC Article

Prognosis

Unmet needs and evidence gaps in hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis.
Wechsler ME, Hellmich B, Cid MC, Jayne D, Tian X, Baylis L, Roufosse F
J Allergy Clin Immunol 2023 Jun;151(6):1415-1428. Epub 2023 Apr 21 doi: 10.1016/j.jaci.2023.03.011. PMID: 37086239
Approach to the patient with suspected hypereosinophilic syndrome.
Klion AD
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):47-54. doi: 10.1182/hematology.2022000367. PMID: 36485140Free PMC Article
Diagnosis and Management of Pediatric Hypereosinophilic Syndrome.
Rosenberg CE, Fulkerson PC, Williams KW
J Allergy Clin Immunol Pract 2022 May;10(5):1131-1138. Epub 2022 Feb 15 doi: 10.1016/j.jaip.2022.02.007. PMID: 35181546
World Health Organization-defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2022 Jan 1;97(1):129-148. Epub 2021 Oct 8 doi: 10.1002/ajh.26352. PMID: 34533850
Hypereosinophilic Syndrome.
Curtis C, Ogbogu P
Clin Rev Allergy Immunol 2016 Apr;50(2):240-51. doi: 10.1007/s12016-015-8506-7. PMID: 26475367

Clinical prediction guides

Unmet needs and evidence gaps in hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis.
Wechsler ME, Hellmich B, Cid MC, Jayne D, Tian X, Baylis L, Roufosse F
J Allergy Clin Immunol 2023 Jun;151(6):1415-1428. Epub 2023 Apr 21 doi: 10.1016/j.jaci.2023.03.011. PMID: 37086239
Living without eosinophils: evidence from mouse and man.
Jackson DJ, Pavord ID
Eur Respir J 2023 Jan;61(1) Epub 2023 Jan 12 doi: 10.1183/13993003.01217-2022. PMID: 35953100Free PMC Article
Clinical Profile and Treatment in Hypereosinophilic Syndrome Variants: A Pragmatic Review.
Requena G, van den Bosch J, Akuthota P, Kovalszki A, Steinfeld J, Kwon N, Van Dyke MK
J Allergy Clin Immunol Pract 2022 Aug;10(8):2125-2134. Epub 2022 Apr 22 doi: 10.1016/j.jaip.2022.03.034. PMID: 35470096
From DREAM to REALITI-A and beyond: Mepolizumab for the treatment of eosinophil-driven diseases.
Pavord ID, Bel EH, Bourdin A, Chan R, Han JK, Keene ON, Liu MC, Martin N, Papi A, Roufosse F, Steinfeld J, Wechsler ME, Yancey SW
Allergy 2022 Mar;77(3):778-797. Epub 2021 Sep 16 doi: 10.1111/all.15056. PMID: 34402066Free PMC Article
Significance of elevated cobalamin (vitamin B12) levels in blood.
Ermens AA, Vlasveld LT, Lindemans J
Clin Biochem 2003 Nov;36(8):585-90. doi: 10.1016/j.clinbiochem.2003.08.004. PMID: 14636871

Recent systematic reviews

What we have learned about lymphocytic variant hypereosinophilic syndrome: A systematic literature review.
Shi Y, Wang C
Clin Immunol 2022 Apr;237:108982. Epub 2022 Mar 17 doi: 10.1016/j.clim.2022.108982. PMID: 35307610
Mepolizumab in Hypereosinophilic Syndrome: A Systematic Review and Meta-analysis.
Alves Júnior JM, Prota FE, Villagelin D, Bley F, Bernardo WM
Clinics (Sao Paulo) 2021;76:e3271. Epub 2021 Oct 11 doi: 10.6061/clinics/2021/e3271. PMID: 34644737Free PMC Article
Endomyocardiofibrosis: A Systematic Review.
Scatularo CE, Posada Martínez EL, Saldarriaga C, Ballesteros OA, Baranchuk A, Sosa Liprandi A, Wyss F, Sosa Liprandi MI
Curr Probl Cardiol 2021 Apr;46(4):100784. Epub 2020 Dec 26 doi: 10.1016/j.cpcardiol.2020.100784. PMID: 33418479
Gleich syndrome: a systematic review.
Haber R, Chebl JA, El Gemayel M, Salloum A
Int J Dermatol 2020 Dec;59(12):1458-1465. Epub 2020 Jun 18 doi: 10.1111/ijd.14963. PMID: 32557651
Causes of eosinophilic ascites - A systematic review.
Pinte L, Baicuş C
Rom J Intern Med 2019 Jun 1;57(2):110-124. doi: 10.2478/rjim-2018-0041. PMID: 30864403

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