Search Database ICD-O-3 Code Lists

Name

Acute myeloid leukemia with MECOM rearrangement (ICD-O-4: 98690/3)

ICD-O-3 Morphology

9869/3: Acute myeloid leukemia with inv(3)(q21;q26.2) or t(3;3)(q21;q26.2); RPN1-EVI1
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

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9861/3.)

Acute myeloid leukemia with MECOM rearrangement 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)

Blood and bone marrow are always involved.

Patients with chronic myeloid leukemia (CML) may acquire MECOM rearrangement; such cases are defined as blast phase regardless of the number of blasts. Although rare, cases with concurrent BCR::ABL1 and MECOM rearrangement at presentation are best regarded as blast phase CML.

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 must be coded as diagnostic confirmation code 3 until the 2027 updates are implemented in your software. Once the software updates are implemented: Assign code 1 for histological confirmation WITH positive genetics

Module Rule

None

Alternate Names

Acute myeloid leukemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1::EVI1 (ICD-O-4: 98690/3)
Acute myeloid leukemia with EVI1 rearrangement (ICD-O-4: 98690/3)

Definition

Acute myeloid leukemia (AML) with MECOM rearrangement is a myeloid neoplasm characterized by rearrangements involving the MDS1 and EVI1 complex locus gene (MECOM). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1-EVI1; MECOM

Immunophenotyping

Not applicable, genetics required for diagnosis

Treatments

Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

Transformations from

Same Primaries

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

C92.0 Acute myeloid leukemia

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

C92.0 Acute myeloblastic leukemia (effective October 01, 2015)
C92.00 Acute myeloblastic leukemia not having achieved remission (effective October 01, 2024)
C92.01 Acute myeloblastic leukemia, in remission (effective October 01, 2024)
C92.02 Acute myeloblastic leukemia, in remission (effective October 01, 2024)

Signs and Symptoms

Anemia
Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Splenomegaly
Thrombocytopenia
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: 1-2% of all AML cases
Sex: no male or female predominance
Survival: aggressive neoplasm with short survival

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: 130-132

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
Glossary