Name

Acute megakaryoblastic leukemia

ICD-O-1 Morphology

9910/3: Acute megakaryoblastic leukemia
Effective 1978 - 1991

ICD-O-2 Morphology

9910/3: Acute megakaryoblastic leukemia
Effective 1992 - 2000

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)

Abstractor Notes

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5:PH10

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.

Grade

Not Applicable

Module Rule

None

Alternate Names

AMKL
FAB M7
Megakaryocytic leukemia

Definition

Acute megakaryoblastic leukemia is an acute leukemia with greater than or equal to 20% blasts, of which greater than or equal to 50% are of megakaryocyte lineage.

Definitive Diagnostic Methods

Bone marrow biopsy
Immunophenotyping
Peripheral blood smear

Genetics Data

None

Immunophenotyping

CD7 aberrant expression
CD13 positive
CD33 positive
CD34 negative
CD36 positive
CD41 (glycoprotein IIb/IIIa) expression
CD42b (glycoprotein Ib) expression
CD45 negative
CD61 (glycoprotein IIIa) expression
HLA-DR negative
MPO negative

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

Transformations to

None

Corresponding ICD-9 Codes

207.2 Megakaryocytic leukemia

Corresponding ICD-10 Codes

C94.2 Acute megakaryoblastic leukemia

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

C94.2 Acute megakaryoblastic leukemia (effective October 01, 2015)

Signs and Symptoms

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

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Reverse transcription-polymerase chain reaction (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 162-164

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary