KEGG   DISEASE: Hypereosinophilic syndromeHelp
Entry
H01599                      Disease                                

Name
Hypereosinophilic syndrome
Description
Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders characterized by marked blood or tissue eosinophilia resulting in a wide variety of clinical manifestations. A number of HES subtypes, or variants, have recently been identified. The best characterized of the clonal molecular defects implicated in myeloproliferative variant HES is the 800-kb interstitial deletion on chromosome 4q12 resulting in the fusion of 2 distinct genes, Fip1-like 1 (FIP1L1) and platelet-derived growth factor receptor-a (PDGFRA), leading to the FIP1L1-PDGFRA fusion kinase. PDGFR is a membrane-bound tyrosine kinase receptor and the fusion PDGFRA kinase has constitutive/unregulated tyrosine kinase activity. The lymphocytic HES variant is associated with T-cell clones producing interleukin-5 (IL-5) and can evolve into lymphoma. While myeloproliferative and lymphocytic HES are well established and permit elimination of the term, idiopathic, to these varieties, most HES patients do not fall into these categories and are classified as complex.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H01599  Hypereosinophilic syndrome
Human diseases in ICD-10 classification [BR:br08403]
 2. Neoplasms (C00-D48)
  D37-D48  Neoplasms of uncertain or unknown behaviour
   D47  Other neoplasms of uncertain or unknown behaviour of lymphoid, haematopoietic and related tissue
    H01599  Hypereosinophilic syndrome
BRITE hierarchy
Gene
FIP1L1-PDGFRA (deletion) [HSA:81608 5156] [KO:K14405 K04363]
Carcinogen
Penicillins [DG:DG01480]
Cephalosporins [DG:DG01488]
Quinolones [DG:DG01550]
Sulfonamides [DG:DG01690]
NSAIDs
Phenytoin [DR:D00512]
Valproate [DR:D00710]
Fluoxetine [DR:D00326]
Amitriptyline [DR:D07448]
Anti-hypertensives (ACE inhibitors, beta blockers)
Pathogen
Parasitic (helminths, ectoparasites, Isospora, Sarcocystis)
Fungal (coccidiomycosis)
Human immunodeficiency virus 1 (HIV-1) [GN:T40001]
Human T-lymphotropic virus 1 [GN:T40003]
Mycobacterium tuberculosis [GN:mtu mtc mra mtf mtb mtg mti mte mtl mtj mtuh mtuc mtue]
Marker
FIP1L1-PDGFRA [HSA:81608 5156] [KO:K14405 K04363]
Drug
Prednisone [DR:D00473]
Hydroxyurea [DR:D00341]
Interferon-alpha [DR:D03305]
Imatinib mesylate [DR:D01441]
Mepolizumab [DR:D04923]
Alemtuzumab [DR:D02802]
Comment
ICD-O-3: 9964/3 Chronic eosinophilic leukemia, NOS
Other DBs
ICD-10: 
MeSH: 
OMIM: 
Reference
PMID:26475367 (gene, env_factor, drug)
  Authors
Curtis C, Ogbogu P
  Title
Hypereosinophilic Syndrome.
  Journal
Clin Rev Allergy Immunol 50:240-51 (2016)
DOI:10.1007/s12016-015-8506-7
Reference
PMID:24726650 (description, gene, drug)
  Authors
Hsieh FH
  Title
Hypereosinophilic syndrome.
  Journal
Ann Allergy Asthma Immunol 112:484-8 (2014)
DOI:10.1016/j.anai.2014.03.004
Reference
PMID:19630574 (description, drug)
  Authors
Klion A
  Title
Hypereosinophilic syndrome: current approach to diagnosis and treatment.
  Journal
Annu Rev Med 60:293-306 (2009)
DOI:10.1146/annurev.med.60.062107.090340
Reference
PMID:16184589 (drug)
  Authors
Wilkins HJ, Crane MM, Copeland K, Williams WV
  Title
Hypereosinophilic syndrome: an update.
  Journal
Am J Hematol 80:148-57 (2005)
DOI:10.1002/ajh.20423
Reference
PMID:19243381 (description, gene, drug)
  Authors
Gleich GJ, Leiferman KM
  Title
The hypereosinophilic syndromes: current concepts and treatments.
  Journal
Br J Haematol 145:271-85 (2009)
DOI:10.1111/j.1365-2141.2009.07599.x
Reference
PMID:20071982 (gene, marker, drug)
  Authors
Gotlib J
  Title
Eosinophilic myeloid disorders: new classification and novel therapeutic strategies.
  Journal
Curr Opin Hematol 17:117-24 (2010)
DOI:10.1097/MOH.0b013e3283366c70

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