| Medical Dictionary: hy·per·e·o·sin·o·phil·ic syndrome |
A syndrome in which the concentration of eosinophils increases in peripheral blood with later infiltration into bone marrow, heart, and other organ systems, accompanied by nocturnal sweating, coughing, anorexia, weight loss, itching, skin lesions, and symptoms of Löffler's endocarditis.
| 5min Related Video: Hypereosinophilic syndrome |
| Veterinary Dictionary: hypereosinophilic syndrome |
A group of disorders in humans characterized by greatly increased numbers of eosinophils in the blood, mimicking leukemia, and eosinophilic infiltration of many tissues. Eosinophilic enteritis, eosinophilic leukemia, and eosinophilic granuloma complex in cats are similar disorders.
| Wikipedia: Hypereosinophilic syndrome |
| Hypereosinophilic syndrome | |
|---|---|
| Classification and external resources | |
 An eosinophil, the white blood cell involved in hypereosinophilic syndrome, seen amongst red blood cells. |
|
| ICD-10 | D72.1 (ILDS D72.12) |
| ICD-9 | 288.3 |
| ICD-O: | 9964/3 |
| OMIM | 607685 |
| eMedicine | med/1076 derm/920 |
| MeSH | [1] |
The hypereosinophilic syndrome (HS) 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.[1]
HS is a diagnosis of exclusion, after clonal eosinophilia (such as leukemia) and reactive eosionophilia (in response to infection, autoimmune disease, atopy, hypoadrenalism or cancer) have been ruled out. [2]
There are some associations with chronic eosinophilic leukemia[3] as it shows similar characteristics and genetic defects.[4]
If left untreated, HS is progressively fatal. It is treated with glucocorticoids such as prednisone.[2] The addition of the monoclonal antibody mepolizumab may reduce the dose of glucorticoids.[5]
Contents |
In the heart, there are two forms of the hypereosinophilic syndrome, endomyocardial fibrosis and Loeffler's endocarditis.
As HS affects many organs at the same time, symptoms may be numerous. Some possible symptoms a patient may present with include:
Numerous techniques are used to diagnose hypereosinophilic syndrome, of which the most important is blood testing. In HS, the eosinophil count is greater than 1.5 × 109/L.[4] On some smears the eosinophils may appear normal in appearance, but morphologic abnormalities, such as a lowering of granule numbers and size, can be observed.[4] Roughly 50% of patients with HS also have anaemia.[4]
Secondly, various imaging and diagnostic technological methods are utilised to detect defects to the heart and other organs, such as valvular dysfunction and arrhythmias by usage of echocardiography.[4] Chest radiographs may indicate pleural effusions and/or fibrosis[4], and neurological tests such as CT scans can show strokes and increased cerebrospinal fluid pressure.[4]
A proportion of patients have a mutation involving the PDGFRA and FIP1L1 genes on the fourth chromosome, leading to a tyrosine kinase fusion protein. Testing for this mutation is now routine practice, as its presence indicates response to imatinib, a tyrosine kinase inhibitor.[8]
Treatment primarily consists of reducing eosinophil levels and preventing further damage to organs.[4] Corticosteroids, such as Prednisone, are good for reducing eosinophil levels and antineoplastics are useful for slowing eosinophil production.[4] Surgical therapy is rarely utilised, however splenectomy can reduce the pain due to spleen enlargement.[4] If damage to the heart (in particular the valves, then prosthetic valves can replace the current organic ones.[4] Follow-up care is vital for the survival of the patient, as such the patient should be checked for any signs of deterioration regularly.[4] After promising results in drug trials (95% efficiency in reducing blood eosinophil count to acceptable levels) it is hoped that in the future hypereosinophilic syndrome, and diseases related to eosinophils such as asthma and Churg-Strauss syndrome, may be treated with the monoclonal antibody Mepolizumab currently being developed to treat the disease.[5] If this becomes successful, it may be possible for corticosteroids to be eradicated and thus reduce the amount of side effects encountered.[5]
HS is very rare, with only 50 cases being noted and followed up in the United States between 1971 and 1982.[4] The disease is even more uncommon within the paediatric population.[4]
Patients which lack chronic heart failure and those who respond well to Prednisone or a similar drug have a good prognosis.[4] However, the mortality rate rises in patients with anaemia, chromosomal abnormalities or a very high white blood cell count.[4]
|
||||||||||||||||||||||||
|
|||||||||||||||||||||||||||||||||||||||||
This entry is from Wikipedia, the leading user-contributed encyclopedia. It may not have been reviewed by professional editors (see full disclaimer)
 | HES |
| Hypereosinophilia |
| Acute eosinophilic leukemia |
 | What is Pickwickian Syndrome? Read answer... |
| What is Moses Syndrome? Read answer... |
| Who has down syndrome? Read answer... |
 | What is Auriculotemporal syndrome? |
| What is adh syndrome? |
| What is the Thalamic Syndrome? |
Copyrights:
 |
 | Medical Dictionary. The American Heritage® Stedman's Medical Dictionary Copyright © 2002, 2001, 1995 by Houghton Mifflin Company. Read more |
 |
| Veterinary Dictionary. Saunders Comprehensive Veterinary Dictionary 3rd Edition. Copyright © 2007 by D.C. Blood, V.P. Studdert and C.C. Gay, Elsevier. All rights reserved. Read more |
 |
| Wikipedia. This article is licensed under the Creative Commons Attribution/Share-Alike License. It uses material from the Wikipedia article "Hypereosinophilic syndrome". Read more |
Mentioned in
