Search Database ICD-O-3 Code Lists

Name

Chronic eosinophilic leukemia (CEL)

ICD-O-3 Morphology

9964/3: Chronic eosinophilic leukemia, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421). Blood and bone marrow are always involved. Damage to a number of organs: heart, lungs, central nervous system (CNS), skin, gastrointestinal tract, spleen and liver are common.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Chronic eosinophilic leukemia (CEL) is part of the Myeloproliferative neoplasms (MPN) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B2)

CEL is a systemic multiorgan disorder. The peripheral blood and bone marrow are always involved; splenic and hepatic involvement is common. Tissue infiltration by eosinophils and the release of cytokines and humoral factors from the eosinophil granules may lead to tissue damage in a number of organs, particularly the heart, lungs, CNS, skin, and gastrointestinal tract

For a diagnosis of CEL, NOS to be made, there should be evidence for clonality of myeloid cells or an increase in myeloblasts in the peripheral blood or bone marrow.

In many cases, it is impossible to prove clonality; in such cases, providing there is no increase in blast cells, the diagnosis of idiopathic hypereosinophilic syndrome (HES) is made.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Hypereosinophilic (idiopathic) syndrome (HES)

Definition

"Chronic eosinophilic leukemia (CEL) is a mye­loproliferative neoplasm (MPN) characterized by an autonomous, clonal proliferation of eosinophil precursors, resulting in persistent eosinophilia in the blood and bone marrow." (WHO 5th edition).

Definitive Diagnostic Methods

Clinical diagnosis

Genetics Data

None

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Immunotherapy
Surgery

Transformations to

Transformations from

None

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

D47.1 Chronic myeloproliferative disease

Corresponding ICD-10-CM Codes (U.S. only)

D47.1 Chronic myeloproliferative disease (effective October 01, 2015 - September 30, 2020)
D72.110 Hypereosonophilic syndrome [HES] (effective October 01, 2020)
D72.111 Lymphocytic Variant Hypereosonophilic Syndrome [LHES] (effective October 01, 2020)
D72.118 Other Hypereosonophilic syndrome (effective October 01, 2020)
D72.119 Hypereosonophilic syndrome (effective October 01, 2020)

Signs and Symptoms

Angioedema (rapid swelling of the dermis)
Cough
Diarrhea
Endomyocardial fibrosis
Fatigue
Fever
Muscle pains
Night sweats
Pruritus
Weight loss

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) Scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

None

Epidemiology and Mortality

Age: median age in the 60's
Incidence: Rare neoplasm (true incidence unknown)
Sex: Male dominance
Survival: 5 year survival about 80% (variable)

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Myeloproliferative neoplasms
Pages: Part A: 37-39

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Myeloproliferative Neoplasms Treatment. Bethesda, MD: National Cancer Institute. Updated <09/27/24>. Available at: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment. Accessed <01/22/25>. [PMID: 26389291]
Section: General Information About Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq
Glossary