KEGG   DISEASE: Chronic eosinophilic leukemiaHelp
Entry
H01590                      Disease                                

Name
Chronic eosinophilic leukemia
Description
Chronic eosinophilic leukemia (CEL) is a chronic myeloproliferative disease of unknown etiology in which a clonal proliferation of eosinophilic precursors results in a persistently elevated number of eosinophils in blood, bone marrow or peripheral tissues. In most patients with CEL (about 60%), eosinophils display PDGFRA-fusion genes and related cytogenetic defects. The most commonly detected oncoprotein is FIP1L1/PDGFRA. FIP1L1-PDGFRA is a constitutively activated tyrosine kinase, which is suggested to induce the proliferation of EoL-1 (The eosinophilic leukemia cell line) cells by inducing c-Myc expression at the mRNA level via ERK and JNK signaling pathways.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H01590  Chronic eosinophilic leukemia
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Myeloproliferative neoplasms
    2A20  Non mast cell myeloproliferative neoplasms
     H01590  Chronic eosinophilic leukemia
BRITE hierarchy
Gene
FIP1L1-PDGFRA (deletion) [HSA:5156] [KO:K04363]
BCR-PDGFRA (translocation) [HSA:5156] [KO:K04363]
Drug
Imatinib mesylate [DR:D01441] (FIP1L1-PDGFRA positive)
Comment
ICD-O-3:9964/3 Chronic eosinophilic leukemia, NOS
Other DBs
ICD-11: 2A20.3
ICD-10: D47.5
MeSH: C580364
Reference
  Authors
Gotlib J, Cools J, Malone JM 3rd, Schrier SL, Gilliland DG, Coutre SE
  Title
The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management.
  Journal
Blood 103:2879-91 (2004)
DOI:10.1182/blood-2003-06-1824
Reference
  Authors
Kumar A, Sinha S, Tripathi AK
  Title
Chronic eosinophilic leukemia: a case report and review of literature.
  Journal
Indian J Hematol Blood Transfus 23:112-5 (2007)
DOI:10.1007/s12288-008-0010-2
Reference
  Authors
Yamada Y, Rothenberg ME, Cancelas JA
  Title
Current concepts on the pathogenesis of the hypereosinophilic syndrome/chronic eosinophilic leukemia.
  Journal
Transl Oncogenomics 1:53-63 (2006)
Reference
PMID:16781488 (gene, drug)
  Authors
Gotlib J, Cross NC, Gilliland DG
  Title
Eosinophilic disorders: molecular pathogenesis, new classification, and modern therapy.
  Journal
Best Pract Res Clin Haematol 19:535-69 (2006)
DOI:10.1016/j.beha.2005.07.013
Reference
PMID:19246139 (description, marker)
  Authors
Valent P
  Title
Pathogenesis, classification, and therapy of eosinophilia and eosinophil disorders.
  Journal
Blood Rev 23:157-65 (2009)
DOI:10.1016/j.blre.2009.01.001
Reference
  Authors
Ishihara K, Kitamura H, Hiraizumi K, Kaneko M, Takahashi A, Zee O, Seyama T, Hong J, Ohuchi K, Hirasawa N
  Title
Mechanisms for the proliferation of eosinophilic leukemia cells by FIP1L1-PDGFRalpha.
  Journal
Biochem Biophys Res Commun 366:1007-11 (2008)
DOI:10.1016/j.bbrc.2007.12.063
Reference
  Authors
De Keersmaecker K, Cools J
  Title
Chronic myeloproliferative disorders: a tyrosine kinase tale.
  Journal
Leukemia 20:200-5 (2006)
DOI:10.1038/sj.leu.2404064

» Japanese version

DBGET integrated database retrieval system