| H01590 | |
| H number | H01590 |
| 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 |
| Network | - |
| Gene | FIP1L1-PDGFRA (deletion) [HSA:5156] [KO:K04363] BCR-PDGFRA (translocation) [HSA:5156] [KO:K04363] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Imatinib mesylate [DR:D01441] (FIP1L1-PDGFRA positive) |
| Comment | - |
| Other DBs | ICD-11: 2A20.3 ICD-10: D47.5 MeSH: C580364 |
| Reference | PMID:15070659 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 PMID:23100928 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 PMID:23662039 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) PMID:16781488 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 PMID:19246139 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 PMID:18086564 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 PMID:16341034 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 |