Back متلازمة فرط اليوزينيات Arabic Hypereosinophilie-Syndrom German Síndrome hipereosinofílico Spanish Síndrome hipereosinofílica idiopática Galician Sindrom Hiper-Eosinofilik Idiopatik ID Sindrome da ipereosinofilia Italian 慢性好酸球性白血病/特発性好酸球増加症候群 Japanese Zespół hipereozynofilowy Polish Síndrome hipereosinofílica Portuguese Хипереозинофилни синдром Serbian
| Hypereosinophilic syndrome | |
|---|---|
| Other names | HES.[1] |
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| Activated eosinophils in the peripheral blood of a patient with idiopathic hypereosinophilic syndrome showing cytoplasmic clearing, nuclear dysplasia, and the presence of immature forms. | |
| Specialty | Hematology |
| Symptoms | Fatigue, breathlessness, cough, muscle pain, fever, and rash.[2] |
| Usual onset | 20-50 years old.[3] |
| Types | Primary (or neoplastic) HES, Secondary (or reactive) HES, and Idiopathic HES.[3] |
| Diagnostic method | Blood chemistries.[3] |
| Differential diagnosis | Acute eosinophilic leukemia, chronic myeloid leukemia, chronic myelomonocytic leukemia, and systemic mastocytosis with eosinophilia.[3] |
| Treatment | Corticosteroids, Imatinib, medications to control eosinophil counts, and supportive care.[4] |
| Frequency | 0.36 to 6.3 per 100,000.[3] |
Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow.[5]
Hypereosinophilic syndrome can manifest in many different ways from nonspecific symptoms and fatigue to neurological impairment and endomyocardial fibrosis, which may be fatal.[6]
There are three different variants of hypereosinophilic syndrome, myeloproliferative, lymphocytic, and idiopathic.[7]
HES is a diagnosis of exclusion, after clonal eosinophilia (such as FIP1L1-PDGFRA-fusion induced hypereosinophelia and leukemia) and reactive eosinophilia (in response to infection, autoimmune disease, atopy, hypoadrenalism, tropical eosinophilia, or cancer) have been ruled out.[8][9]
There are some associations with chronic eosinophilic leukemia[10] as it shows similar characteristics and genetic defects.[11] If left untreated, HES is progressive and fatal. It is treated with glucocorticoids such as prednisone.[8] The addition of the monoclonal antibody mepolizumab may reduce the dose of glucocorticoids.[12]
- ^ "Monarch Initiative". Monarch Initiative. Retrieved February 6, 2024.
- ^ "Symptoms and causes". Mayo Clinic. April 27, 2022. Retrieved February 6, 2024.
- ^ a b c d e "UpToDate". UpToDate. Retrieved February 6, 2024.
- ^ Liesveld, Jane (January 4, 2024). "Hypereosinophilic Syndrome". Merck Manuals Professional Edition. Retrieved February 6, 2024.
- ^ Chusid MJ, Dale DC, West BC, Wolff SM (1975). "The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature". Medicine (Baltimore). 54 (1): 1–27. doi:10.1097/00005792-197501000-00001. PMID 1090795. S2CID 39212252.
- ^ Weller, PF; Bubley, GJ (May 15, 1994). "The idiopathic hypereosinophilic syndrome". Blood. 83 (10). American Society of Hematology: 2759–2779. doi:10.1182/blood.v83.10.2759.2759. ISSN 0006-4971.
- ^ Roufosse, Florence; Cogan, Elie; Goldman, Michel (2003). "The Hypereosinophilic Syndrome Revisited". Annual Review of Medicine. 54 (1). Annual Reviews: 169–184. doi:10.1146/annurev.med.54.101601.152431. ISSN 0066-4219. PMID 12525672.
- ^ a b Fazel R, Dhaliwal G, Saint S, Nallamothu BK (May 2009). "Clinical problem-solving. A red flag". N. Engl. J. Med. 360 (19): 2005–10. doi:10.1056/NEJMcps0802754. PMID 19420370.
- ^ Reiter A, Gotlib J (2017). "Myeloid neoplasms with eosinophilia". Blood. 129 (6): 704–714. doi:10.1182/blood-2016-10-695973. PMID 28028030.
- ^ Longmore, Murray; Ian Wilkinson; Tom Turmezei; Chee Kay Cheung (2007). Oxford Handbook of Clinical Medicine. Oxford. p. 316. ISBN 978-0-19-856837-7.
- ^ Rothenberg, Marc E (2008). "Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab". The New England Journal of Medicine. 358 (12): 1215–28. doi:10.1056/NEJMoa070812. PMID 18344568. S2CID 6037384. Retrieved 2008-03-17. Last updated: Updated: Oct 4, 2009 by Venkata Samavedi and Emmanuel C Besa
- ^ Rothenberg ME, Klion AD, Roufosse FE, et al. (March 2008). "Treatment of patients with the hypereosinophilic syndrome with mepolizumab". N. Engl. J. Med. 358 (12): 1215–28. doi:10.1056/NEJMoa070812. PMID 18344568. S2CID 6037384.