Hypereosinophilic syndrome: Difference between revisions

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A heterogeneous group of disorders with the common feature of prolonged [[eosinophilia]] of unknown cause and associated organ system dysfunction, including the [[heart]], [[central nervous system]], [[kidney]]s, [[lungs]], [[gastrointestinal tract]], and [[skin]]. {<ref name=MeSH>{{MeSH}}</ref> There is an [[idiopathic]] form not otherwise characterized, but the group also includes [[Churg-Strauss syndrome]], and a group of  Eosinophil-Associated Gastrointestinal Disorders (EGID):<ref>{{citation
A heterogeneous group of disorders with the common feature of prolonged [[eosinophilia]] of unknown cause and associated organ system dysfunction, including the [[heart]], [[central nervous system]], [[kidney]]s, [[lung]]s, [[gastrointestinal tract]], and [[skin]]. {<ref name=MeSH>{{MeSH}}</ref> There is an [[idiopathic]] form not otherwise characterized, but the group also includes [[Churg-Strauss syndrome]], and a group of  Eosinophil-Associated Gastrointestinal Disorders (EGID):<ref>{{citation
  | url = http://www.apfed.org/egid.htm
  | url = http://www.apfed.org/egid.htm
  | publisher = American Partnership for Eosinophilic Disorders
  | publisher = American Partnership for Eosinophilic Disorders
  | title = About EGID}}</ref>
  | title = About EGID}}</ref>
*Eosinophilic Esophagitis (EE): high numbers of eosinophils occurring in the esophagus.    Learn more: *Eosinophilic Esophagitis
*Eosinophilic Esophagitis (EE): high numbers of eosinophils occurring in the esophagus.    *Eosinophilic Esophagitis
*Eosinophilic Gastritis (EG): high numbers of eosinophils in the stomach.
*Eosinophilic Gastritis (EG): high numbers of eosinophils in the stomach.
*Eosinophilic Gastroenteritis (EGE): affects the stomach and small intestine.
*Eosinophilic Gastroenteritis (EGE): affects the stomach and small intestine.
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  | journal = eMedicine
  | journal = eMedicine
  | url = http://emedicine.medscape.com/article/1051555-overview}}</ref>
  | url = http://emedicine.medscape.com/article/1051555-overview}}</ref>
*a peripheral eosinophil count of greater than 1.5 X 109/L for longer than 6 months
*a peripheral eosinophil count of greater than 1.5 per microliter for longer than 6 months
*evidence of organ involvement, thus excluding benign eosinophilia
*evidence of organ involvement, thus excluding benign eosinophilia
*an absence of other causes of eosinophilia, such as parasite infestation (most common cause of eosinophilia worldwide), allergy (most common cause of eosinophilia in the United States), malignancy, and collagen-vascular disease.
*an absence of other causes of eosinophilia, such as parasite infestation (most common cause of eosinophilia worldwide), allergy (most common cause of eosinophilia in the United States), malignancy, and collagen-vascular disease.
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  | title = Idiopathic Eosinophilia}}</ref>
  | title = Idiopathic Eosinophilia}}</ref>
==Pathology==
==Pathology==
There is a massive increase in the number of  [[eosinophil]]s in the blood, mimicking [[leukemia]], and extensive eosinophilic infiltration of the various organs. <ref name=MeSH/> Eosinophil production is known to be stimulated by [[interleukins]].
There is a massive increase in the number of  [[eosinophil]]s in the blood, mimicking [[leukemia]], and extensive eosinophilic infiltration of the various organs. <ref name=MeSH/> Eosinophil production is known to be stimulated by [[interleukin]]s.<ref>{{citation
| journal = International Immunology
| year = 2010
  | volume = 22 | issue = 6 | pages = 479-489| doi= 10.1093/intimm/dxq035
| title = Contribution of IL-33 to induction and augmentation of experimental allergic conjunctivitis
| author = Saori Matsuba-Kitamura ''et al''
| url =http://intimm.oxfordjournals.org/cgi/content/full/22/6/479
}}</ref>
==References==
==References==
{{reflist}}
{{reflist|2}}

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A heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and associated organ system dysfunction, including the heart, central nervous system, kidneys, lungs, gastrointestinal tract, and skin. {[1] There is an idiopathic form not otherwise characterized, but the group also includes Churg-Strauss syndrome, and a group of Eosinophil-Associated Gastrointestinal Disorders (EGID):[2]

  • Eosinophilic Esophagitis (EE): high numbers of eosinophils occurring in the esophagus. *Eosinophilic Esophagitis
  • Eosinophilic Gastritis (EG): high numbers of eosinophils in the stomach.
  • Eosinophilic Gastroenteritis (EGE): affects the stomach and small intestine.
  • Eosinophilic Colitis (EC): describes the occurrence of high numbers of eosinophils in the large intestine.

The diagnosis requires:[3]

  • a peripheral eosinophil count of greater than 1.5 per microliter for longer than 6 months
  • evidence of organ involvement, thus excluding benign eosinophilia
  • an absence of other causes of eosinophilia, such as parasite infestation (most common cause of eosinophilia worldwide), allergy (most common cause of eosinophilia in the United States), malignancy, and collagen-vascular disease.

A support group reports three patients with the idiopathic form. [4]

Pathology

There is a massive increase in the number of eosinophils in the blood, mimicking leukemia, and extensive eosinophilic infiltration of the various organs. [1] Eosinophil production is known to be stimulated by interleukins.[5]

References

  1. 1.0 1.1 Anonymous (2024), Hypereosinophilic syndrome (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. About EGID, American Partnership for Eosinophilic Disorders
  3. Noah S Scheinfeld, Felix Urman (1 February 2010), "eMedicine Specialties > Dermatology > Allergy & Immunology > Hypereosinophilic Syndrome: Introduction", eMedicine
  4. Idiopathic Eosinophilia, Hyper Eosinophilia Syndrome (HES)
  5. Saori Matsuba-Kitamura et al (2010), "Contribution of IL-33 to induction and augmentation of experimental allergic conjunctivitis", International Immunology 22 (6): 479-489, DOI:10.1093/intimm/dxq035