Chronic eosinophilic leukemia

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Chronic eosinophilic leukemia
Other names	CEL
Specialty	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

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

^ "Chronic Eosinophilic Leukemia". The Lecturio Medical Concept Library. Retrieved 8 July 2021.
^ ^a ^b ^c ^d Jaffe, Elaine (2016-08-25). Hematopathology (2 ed.). pp. 931–941. ISBN 978-0-323-29613-7.
^ McPherson (2011). Henry's Clinical Diagnosis and Management by Laboratory Methods (23 ed.). Elsevier. pp. 606–658. ISBN 9781437709742.
^ ^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.
^ 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.

External links

Chronic eosinophilic leukemia entry in the public domain NCI Dictionary of Cancer Terms
Cancer.Net: Eosinophilic Leukemia

CFU-GM/
and other granulocytes

CFU-GM

Myelocyte

AML	Acute myeloblastic leukemia M0 M1 M2 APL/M3
MP	Chronic neutrophilic leukemia

Monocyte

AML	AMoL/M5 Myeloid dendritic cell leukemia
CML	Philadelphia chromosome Accelerated phase chronic myelogenous leukemia

Myelomonocyte

AML	M4
MD-MP	Juvenile myelomonocytic leukemia Chronic myelomonocytic leukemia

Other

Histiocytosis

CFU-Baso

AML	Acute basophilic

CFU-Eos

AML	Acute eosinophilic
MP	Chronic eosinophilic leukemia/Hypereosinophilic syndrome

MEP

CFU-Meg

MP	Essential thrombocythemia Acute megakaryoblastic leukemia

CFU-E

AML	Erythroleukemia/M6
MP	Polycythemia vera
MD	Refractory anemia Refractory anemia with excess of blasts Chromosome 5q deletion syndrome Sideroblastic anemia Paroxysmal nocturnal hemoglobinuria Refractory cytopenia with multilineage dysplasia

CFU-Mast

Mastocytoma	Mast cell leukemia Mast cell sarcoma Systemic mastocytosis
Mastocytosis	Diffuse cutaneous mastocytosis Erythrodermic mastocytosis Adult type of generalized eruption of cutaneous mastocytosis Urticaria pigmentosa Mast cell sarcoma Solitary mastocytoma
Systemic mastocytosis	Xanthelasmoidal mastocytosis

Multiple/unknown

AML	Acute panmyelosis with myelofibrosis Myeloid sarcoma
MP	Primary myelofibrosis Biphenotypic acute leukaemia

Categories:

Hidden categories:

Classification	D ICD-O: 9964/3 MeSH: C580364
External resources	Orphanet: 168940