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 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
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.