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Primary hypereosinophilic syndrome

MedGen UID:
1830089
Concept ID:
C5679898
Disease or Syndrome
Synonyms: Clonal hypereosinophilic syndrome; clonal hypereosinophilic syndrome; HES-M; HES-N; HES-N - hypereosinophilic syndrome neoplastic; Neoplastic hypereosinophilic syndrome; neoplastic hypereosinophilic syndrome; primary HES; Primary HES; Primary HES (hypereosinophilic syndrome); primary hypereosinophilic syndrome
SNOMED CT: Primary hypereosinophilic syndrome (1264010001); Clonal hypereosinophilic syndrome (1264010001); Neoplastic hypereosinophilic syndrome (1264010001); Primary HES (hypereosinophilic syndrome) (1264010001); HES-N - hypereosinophilic syndrome neoplastic (1264010001)
 
Monarch Initiative: MONDO:0017833
Orphanet: ORPHA314950

Definition

A rare syndrome with characteristics of hypereosinophilia produced by clonal eosinophils derived from neoplastic stem cells in the absence of any secondary cause of eosinophilia and persisting for at least six months. The condition is associated with signs of organ infiltration, dysfunction and damage. Clinical manifestations are highly variable depending on the organ systems involved, and include dermatologic, pulmonary, cardiac, gastrointestinal and cerebral manifestations among others. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary hypereosinophilic syndrome

Professional guidelines

PubMed

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
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
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

Recent clinical studies

Etiology

Pagnoux C, Nair P, Xi Y, Khalidi NA, Carette S, Cuthbertson D, Grayson PC, Koening CL, Langford CA, McAlear CA, Moreland LW, Monach PA, Seo P, Specks U, Sreih AG, Ytterberg SR, Tyrrell PN, Merkel PA; Vasculitis Clinical Research Consortium
Clin Exp Rheumatol 2019 Mar-Apr;37 Suppl 117(2):40-44. Epub 2019 Jan 14 PMID: 30652675Free PMC Article
Mittman RJ, Bernstein DI, Steinberg DR, Enrione M, Bernstein IL
J Allergy Clin Immunol 1989 Sep;84(3):304-10. doi: 10.1016/0091-6749(89)90412-0. PMID: 2778236

Diagnosis

Voeller J, DeNapoli T, Griffin TC
Cancer Rep (Hoboken) 2022 Nov;5(11):e1710. Epub 2022 Oct 14 doi: 10.1002/cnr2.1710. PMID: 36241191Free PMC Article
Spina E, Maniscalco GT, Petraroli A, Detoraki A, Servillo G, Ranieri A, De Mase A, Renna R, Candelaresi P, De Paulis A, Andreone V
J Stroke Cerebrovasc Dis 2022 Nov;31(11):106703. Epub 2022 Sep 5 doi: 10.1016/j.jstrokecerebrovasdis.2022.106703. PMID: 36075130
Crapé L, Strubbe B, Cesmeli E, de Clerck F
Acta Gastroenterol Belg 2019 Oct-Dec;82(4):532-535. PMID: 31950810
Pagnoux C, Nair P, Xi Y, Khalidi NA, Carette S, Cuthbertson D, Grayson PC, Koening CL, Langford CA, McAlear CA, Moreland LW, Monach PA, Seo P, Specks U, Sreih AG, Ytterberg SR, Tyrrell PN, Merkel PA; Vasculitis Clinical Research Consortium
Clin Exp Rheumatol 2019 Mar-Apr;37 Suppl 117(2):40-44. Epub 2019 Jan 14 PMID: 30652675Free PMC Article
Lincoff NS, Schlesinger D
J Neuroophthalmol 2005 Jun;25(2):116-21. doi: 10.1097/01.wno.0000165318.67686.08. PMID: 15937435

Therapy

Spina E, Maniscalco GT, Petraroli A, Detoraki A, Servillo G, Ranieri A, De Mase A, Renna R, Candelaresi P, De Paulis A, Andreone V
J Stroke Cerebrovasc Dis 2022 Nov;31(11):106703. Epub 2022 Sep 5 doi: 10.1016/j.jstrokecerebrovasdis.2022.106703. PMID: 36075130
Lincoff NS, Schlesinger D
J Neuroophthalmol 2005 Jun;25(2):116-21. doi: 10.1097/01.wno.0000165318.67686.08. PMID: 15937435
Mittman RJ, Bernstein DI, Steinberg DR, Enrione M, Bernstein IL
J Allergy Clin Immunol 1989 Sep;84(3):304-10. doi: 10.1016/0091-6749(89)90412-0. PMID: 2778236

Prognosis

Spina E, Maniscalco GT, Petraroli A, Detoraki A, Servillo G, Ranieri A, De Mase A, Renna R, Candelaresi P, De Paulis A, Andreone V
J Stroke Cerebrovasc Dis 2022 Nov;31(11):106703. Epub 2022 Sep 5 doi: 10.1016/j.jstrokecerebrovasdis.2022.106703. PMID: 36075130

Clinical prediction guides

Pagnoux C, Nair P, Xi Y, Khalidi NA, Carette S, Cuthbertson D, Grayson PC, Koening CL, Langford CA, McAlear CA, Moreland LW, Monach PA, Seo P, Specks U, Sreih AG, Ytterberg SR, Tyrrell PN, Merkel PA; Vasculitis Clinical Research Consortium
Clin Exp Rheumatol 2019 Mar-Apr;37 Suppl 117(2):40-44. Epub 2019 Jan 14 PMID: 30652675Free PMC Article
Mittman RJ, Bernstein DI, Steinberg DR, Enrione M, Bernstein IL
J Allergy Clin Immunol 1989 Sep;84(3):304-10. doi: 10.1016/0091-6749(89)90412-0. PMID: 2778236

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