Chronic eosinophilic leukemia

Chronic eosinophilic leukemia
Other namesCEL
SpecialtyHematology, 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

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]

Causes

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]

Diagnosis

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]

Prognosis

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]

Epidemiology

Cases occur in people of all ages. The disease is more common in males than females.[2]

References

  1. ^ "Chronic Eosinophilic Leukemia". The Lecturio Medical Concept Library. Retrieved 8 July 2021.
  2. ^ a b c d Jaffe, Elaine (2016-08-25). Hematopathology (2 ed.). pp. 931–941. ISBN 978-0-323-29613-7.
  3. ^ McPherson (2011). Henry's Clinical Diagnosis and Management by Laboratory Methods (23 ed.). Elsevier. pp. 606–658. ISBN 9781437709742.
  4. ^ a b c d Hoffman, Ronald (2017-08-15). Hematology: basic principles and practice (Seven ed.). pp. 1151–1169. ISBN 978-0-323-35762-3.
  5. ^ Stonecypher, Mark; Chen, Benjamin J. (2013). "Chronic Eosinophilic Leukemia (CEL)". In Aster, J.C.; Pozdnyakova, O.; Kutok, J.L. (eds.). Hematopathology. High-Yield Pathology. Elsevier. pp. 222–3. doi:10.1016/B978-1-4377-1758-7.00174-3. ISBN 978-1-4377-1758-7.