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a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/10/14 06:42:10」(JST)

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The hypereosinophilic syndrome (HES) 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]

HES is a diagnosis of exclusion, after clonal eosinophilia (such as leukemia) and reactive eosinophilia (in response to infection, autoimmune disease, atopy, hypoadrenalism, tropical eosinophilia, 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, HES is progressively fatal. It is treated with glucocorticoids such as prednisone.^[2] The addition of the monoclonal antibody mepolizumab may reduce the dose of glucocorticoids.^[5]

Classification

In the heart, there are two forms of the hypereosinophilic syndrome, endomyocardial fibrosis and Loeffler's endocarditis.

Endomyocardial fibrosis (also known as Davies disease) is seen in tropical areas.^[6]^[7]
Loeffler's endocarditis does not have any geographic predisposition.

Signs and symptoms

As HES affects many organs at the same time, symptoms may be numerous. Some possible symptoms a patient may present with include:

Cardiomyopathy^[3]
Skin lesions^[3]
Thromboembolic disease^[3]
Pulmonary disease^[3]
Neuropathy^[3]
Hepatosplenomegaly^[3]
Reduced ventricular size^[3]

Diagnosis

Numerous techniques are used to diagnose hypereosinophilic syndrome, of which the most important is blood testing. In HES, 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 HES 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

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]

Epidemiology

HES is very rare, with only 50 cases being noted and followed up in the United States between 1971 and 1982,^[4] corresponding roughly to a prevalence of 1 to 2 per million people. The disease is even more uncommon within the paediatric population.^[4]

Patients who 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]

References

^ 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.
^ ^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.
^ ^a ^b ^c ^d ^e ^f ^g ^h Longmore, Murray; Ian Wilkinson; Tom Turmezei; Chee Kay Cheung (2007). Oxford Handbook of Clinicial Medicine. Oxford. p. 316. ISBN 0-19-856837-1.
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j ^k ^l ^m ⁿ ^o ^p Rothenberg, Marc E. "Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab". Retrieved 2008-03-17. Last updated: Updated: Oct 4, 2009 by Venkata Samavedi and Emmanuel C Besa
^ ^a ^b ^c 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.
^ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 606. ISBN 0-7216-0187-1.
^ Smedema JP, Winckels SK, Snoep G, Vainer J, Bekkers SC, Crijns HJ (December 2004). "Tropical endomyocardial fibrosis (Davies' disease): case report demonstrating the role of magnetic resonance imaging" (PDF). Int J Cardiovasc Imaging 20 (6): 517–22. doi:10.1007/s10554-004-1095-9. PMID 15856635.
^ Cools J, DeAngelo DJ, Gotlib J, et al. (2003). "A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome". N. Engl. J. Med. 348 (13): 1201–14. doi:10.1056/NEJMoa025217. PMID 12660384.

External links

DermNet systemic/hypereosinophilic
Hypereosinophilic Syndrome research in UK
Hypereosinophilic Syndrome on patient.co.uk
Hypereosinophilic Syndrome on eMedicine
Hypereosinophilic Syndrome (HES) on American Academy of Allergy, Asthma & Immunology
Hypereosinophilic syndrome on Mayo Clinic

UpToDate Contents

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1. 好酸球増加症候群の臨床症状、病態生理、および診断 clinical manifestations pathophysiology and diagnosis of the hypereosinophilic syndromes
2. 好酸球増加症候群の治療 treatment of the hypereosinophilic syndromes
3. 原因不明の好酸球増加症患者に対するアプローチ approach to the patient with unexplained eosinophilia
4. 下肢末梢動脈疾患の症状を治療するための試験的治療 investigational therapies for treating symptoms of lower extremity peripheral artery disease
5. 肺好酸球増多症の原因 causes of pulmonary eosinophilia

English Journal

Eosinophilic cystitis in a patient with hypereosinophila syndrome: A case report.

Jiang P, Wang C, Jin B, Lin Y, Chen S.
Experimental and therapeutic medicine.Exp Ther Med.2014 Jul;8(1):49-51. Epub 2014 May 12.
Hypereosinophilic syndrome (HES) is a rare disorder that is characterized by hypereosinophilia and organ damage, caused by the infiltration of eosinophils. In rare cases, the urinary bladder may also be involved. The current case report presented a 56-year-old male with gross hematuria and hypereosi
PMID 24944595

Löffler endocarditis with multiple cerebral embolism.

Wang S1, Wang A1, Guo B1, Zhu S1, Chi Z1, Zhao X2.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association.J Stroke Cerebrovasc Dis.2014 Jul;23(6):1709-12. doi: 10.1016/j.jstrokecerebrovasdis.2013.10.023. Epub 2014 Feb 13.
Idiopathic hypereosinophilic syndrome is an uncommon leukoproliferative systemic disorder characterized by the sustained eosinophilia and target organ damage. We report the case of a 56-year-old man presenting with multiple cerebral embolism, Löffler endocarditis, and hypereosinophilia. This patien
PMID 24529355

Hypereosinophilic syndrome.

Hsieh FH.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.Ann Allergy Asthma Immunol.2014 Jun;112(6):484-8. doi: 10.1016/j.anai.2014.03.004. Epub 2014 Apr 13.
OBJECTIVE: To summarize the identified molecular and cellular mechanisms relevant to clinicians evaluating patients with hypereosinophilic syndrome (HES).DATA SOURCES: Review of relevant peer-reviewed literature.STUDY SELECTIONS: Studies on the pathogenesis of HES in relation to consensus definition
PMID 24726650

Japanese Journal

専門医のためのアレルギー学講座 9.好酸球増多を主徴とする疾患(1)Idiopathic Hypereosinophilic Syndrome (idiopathic HES)

松尾佳美,猪熊茂子
アレルギー 60(1), 1-8, 2011-01-30
NAID 110008440530

IL-5の著明な高値を認めた特発性好酸球増多症の1例

坂本謙一,今村俊彦,塩見梢 [他]
日本小児科学会雑誌 115(1), 97-101, 2011-01
NAID 40018699544

Related Pictures

★リンクテーブル★

リンク元	「イマチニブ」「好酸球増多症候群」「好酸球増加症候群」「Loeffler's endocarditis」「HES」
拡張検索	「chronic eosinophilic leukemia/hypereosinophilic syndrome」「idiopathic hypereosinophilic syndrome」
関連記事	「syndrome」

「イマチニブ」

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
DiseasesDB	34939
eMedicine	article/202030 article/1051555 article/886861
MeSH	D017681

　　[★]

英: imatinib
化: imatinib mesylate
商: グリベック
関: nib; 分子標的薬剤

分類

その他

チロシンキナーゼ阻害薬

概念

低分子のチロシンキナーゼ阻害薬である。
最初はPDGFRに特異的な阻害薬である2-phenylaminopyrimidine誘導体として開発された。
t(9;22)の染色体転座を有する慢性骨髄性白血病でみられるBCR-ABL融合蛋白を含め、ABLキナーゼに対する強い阻害作用を持つことが明らかとなった。
また受容体チロシンキナーゼであるC-KITのキナーゼも阻害することが明らかとなった。
造血幹細胞因子(SCF)の受容体はC-KITであるが、C-KITの変異はGISTやsystemic mastocytosisでしばしば見られる。
FIPL1-PDGFRA融合蛋白質の発現が特徴的なidiopathic hypereosinophilic syndromeはイマチニブ投与によりPDGFRAで阻害することで治療が成功する。

特徴

構造

作用機序

ABLや、活性複合体であるv-ABL, BCR-ABL, [EVT6]-ABLを阻害する (GOO.1367)

(そのほかに)PDGFRとKITに対しても競合阻害作用を持つ (GOO.1367)

KITはgastrointestinal stromal tumor(GIST)と関連

ETV6-PDGFRはchronic myelomonocytic leukemia(CMML)と関連

FIP1L1-PDGFRAはhypereosinophilic syndrome(HES)と関連

薬理作用

動態

経口投与

適応

慢性骨髄性白血病 chronic myelocytic leukemia CML
胃腸間質性腫瘍 astrointestinal stromal tumors
好酸球増多症候群 hypereosinophilic syndrome

グリベック錠100mg

1. 慢性骨髄性白血病
2. KIT陽性消化管間質腫瘍
3. フィラデルフィア染色体陽性急性リンパ性白血病

注意

禁忌

副作用

表在性浮腫、嘔気・嘔吐、筋痙攣、皮疹、下痢

相互作用

類似薬

添付文書

グリベック錠100mg

http://www.info.pmda.go.jp/go/pack/4291011F1028_1_11/4291011F1028_1_11?view=body

「好酸球増多症候群」

　　[★]

同: hypereosinophilic syndrome, HES

英

hypereosinophilic syndrome

hypereosinophilic syndrome

関: 好酸球増加症候群、特発性好酸球増多症候群、好酸球性白血病、レフラー心内膜炎

「好酸球増加症候群」

　　[★]

英: hypereosinophilic syndrome
関: 好酸球増多症候群

-播種性好酸球性膠原病

同: HES

-HES

英: hypereosinophilic syndrome
同: HES

-好酸球性白血病

「Loeffler's endocarditis」

　　[★]

レフラー心内膜炎、Loeffler心内膜炎

関: eosinophilic leukemia、hypereosinophilic syndrome、idiopathic hypereosinophilic syndrome

「HES」

　　[★]

「chronic eosinophilic leukemia/hypereosinophilic syndrome」

　　[★] 慢性好酸球性白血病/好酸球増加症候群

「idiopathic hypereosinophilic syndrome」

　　[★] 特発性好酸球増多症候群

「syndrome」

　　[★]

n.

症候群

[pmid1090795-1] 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.

[Fazel-2] 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.

[oxford-3] ^ ^a ^b ^c ^d ^e ^f ^g ^h Longmore, Murray; Ian Wilkinson; Tom Turmezei; Chee Kay Cheung (2007). Oxford Handbook of Clinicial Medicine. Oxford. p. 316. ISBN 0-19-856837-1.

[emedicine-4] ^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j ^k ^l ^m ⁿ ^o ^p Rothenberg, Marc E. "Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab". Retrieved 2008-03-17. Last updated: Updated: Oct 4, 2009 by Venkata Samavedi and Emmanuel C Besa

[nejm-5] 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.

[isbn0-7216-0187-1-6] Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 606. ISBN 0-7216-0187-1.

[pmid15856635-7] Smedema JP, Winckels SK, Snoep G, Vainer J, Bekkers SC, Crijns HJ (December 2004). "Tropical endomyocardial fibrosis (Davies' disease): case report demonstrating the role of magnetic resonance imaging" (PDF). Int J Cardiovasc Imaging 20 (6): 517–22. doi:10.1007/s10554-004-1095-9. PMID 15856635.

[pmid12660384-8] Cools J, DeAngelo DJ, Gotlib J, et al. (2003). "A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome". N. Engl. J. Med. 348 (13): 1201–14. doi:10.1056/NEJMoa025217. PMID 12660384.

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
DiseasesDB	34939
eMedicine	article/202030 article/1051555 article/886861
MeSH	D017681

匿名

検索

案内