Search Database ICD-O-3 Code Lists

Name

Acute megakaryoblastic leukemia (AMKL)

ICD-O-3 Morphology

9910/3: Acute megakaryoblastic 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 megakaryoblastic leukemia (AMKL) 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. Extramedullary disease may also occur.

Cases in patients with a history of chronic myeloid leukemia (CML) or other forms of myeloproliferative neoplasm that have transformed should be classified as myeloproliferative neoplasm in blast phase.

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

None

Alternate Names

FAB M7
Megakaryocytic leukemia

Definition

Acute megakaryoblastic leukemia (AMKL) is characterized by ≥ 20% blasts with megakaryocytic differentiation. (WHO 5th edition)

Definitive Diagnostic Methods

Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

None

Immunophenotyping

CD33+ (expression/positive)
CD34+ ((expression/positive)
CD36+ (expression/positive)
CD41 (glycoprotein IIb/IIIa)+ (expression/positive)
CD42b (glycoprotein Ib)+ (expression/positive)
CD61 (glycoprotein IIIa)+ (expression/positive)
HLA-DR- (not expression/negative)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

Transformations to

None

Transformations from

Same Primaries

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

C94.2 Acute megakaryoblastic leukemia

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

C94.2 Acute megakaryoblastic leukemia (effective October 01, 2015 - September 30, 2024)
C94.20 Acute megakaryoblastic leukemia not having achieved remission (effective October 01, 2024)
C94.21 Acute megakaryoblastic leukemia, in remission (effective October 01, 2024)
C94.2 Acute megakaryoblastic leukemia, in relapse (effective October 01, 2024)

Signs and Symptoms

Anemia
Asthenia
Cytopenia
Easy bruising or bleeding
Fever
Hepatosplenomegaly
Severe infections (due to decreased neutrophils)
Thrombocytopenia
Thrombocytosis

Diagnostic Exams

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

Progression and Transformation

None

Epidemiology and Mortality

Age: occurs in both children and adults
Incidence: <5% of all AML cases
Survival: poor prognosis

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: 162-164

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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