Search Database ICD-O-3 Code Lists

Name

Acute erythroid leukemia (AEL)

ICD-O-3 Morphology

9840/3: Acute erythroid leukemia
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Acute erythroid leukemia (AEL) is part of the Acute myeloid leukemia (AML) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B6)

This leukemia predominantly involves the peripheral blood and bone marrow.

If this leukemia and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.

If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.

See Multiple Primary Rule M3

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

See abstractor notes

Alternate Names

Acute erythemia [OBS]
Acute erythremic myelosis [OBS]
Acute myeloid leukemia, M6 (AML M6)
Acute myeloid leukemia, M6 type
Acute myeloid leukemia, M6a (AML M6a)
Acute myeloid leukemia, M6b (AML M6b)
Di Guglielmo disease [OBS]
Erythremic myelosis, NOS
Erythroleukemia
FAB M6
Pure erythroid leukemia

Definition

Acute erythroid leukemia (AEL) is a neoplastic proliferation of erythroid cells with features of maturation arrest (increased proerythroblasts) and a high prevalence of biallelic TP53 alterations. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Immunophenotyping

CD71+ (expression/positive) (pan erythroid marker)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

Transformations from

Same Primaries

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

C94.0 Acute erythremia and erythroleukemia

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

C94.0 Acute erythroid leukemia (effective October 01, 2015 - September 30, 2024)
C94.00 Acute erythroid leukemia not having achieved remission (effective October 01, 2024)
C94.01 Acute erythroid leukemia, in remission (effective October 01, 2024)
C94.02 Acute erythroid leukemia, in relapse (effective October 01, 2024)

Signs and Symptoms

Anemia
Circulating erythroblasts
Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite

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: predominantly adults
Incidence: <5% of all AML cases
Survival: poor, aggressive clinical course

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: Acute myeloid leukemia
Pages: Part A: 160-161

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 Acute Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <03/06/2024>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389432]
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
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