Chronic eosinophilic leukemia | |
Synonyms: | CEL |
Field: | hematology, oncology |
Chronic eosinophilic leukemia is a form of cancer in which too many eosinophils are found in the bone marrow, blood, and other tissues. Most cases are associated with fusion genes. [1]
Signs and symptoms may include weight loss, fever, malaise, cough, skin and mucosal lesions, diarrhea, and peripheral neuropathy. Cardiac symptoms are also possible.[2]
In cases associated with PDGFRB and FGFR1 mutations, splenomegaly is common. Lymphadenopathy is also common with FGFR1 mutations.[2]
Infiltration of eosinophils causes organ damage.[3]
Most cases of CEL are associated with rearrangements in PDGFRA, PDGFRB, or FGFR1.[4]
CEL not otherwise specified (CEL NOS) is a form in which BCR-ABL1 fusion genes and PDGFRA, PDGFRB, and FGFR1 rearrangements are not found.[5]
For a diagnosis of CEL, hypereosinophilia with greater than 30% eosinophils is required.[4] Serum IgE is usually normal.In cases associated with PDGFRB, serum vitamin B12 and tryptase may be elevated.[2]
CEL associated with a mutation in PDGFRA is treatable with imatinib and has an excellent prognosis. On the other hand, CEL associated with FGFR1 mutations has a very poor prognosis.[4] Progression can occur from CEL to AEL or AML in rare cases.[4]
Cases occur in people of all ages. The disease is more common in males than females.[2]