Name

Acute myeloid leukemia with BCR-ABL1

ICD-O-3 Morphology

9912/3: Acute myeloid leukemia with BCR-ABL1
Effective 2021 and later

Reportable

for cases diagnosed 2021 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9861/3.)

Patients most commonly present with leukocytes with a blast predominance and variable presence of anemia and thrombocytopenia.

This is an aggressive disease, with poor response to traditional AML therapy or tyrosine kinase inhibitor therapy alone. Improved survival is seen with tyrosine kinase inhibitor followed by allogeneic hematopoietic cell transplantation.

Grade

Not Applicable

Module Rule

None

Alternate Names

AML with BCR-ABL1

Definition

Acute myeloid leukemia (AML) with BCR-ABL1 (a provisional entity in the current classification) is a de novo AML in which patients show no evidence (either before or after therapy) of chronic myeloid leukemia (CML). Cases that meet the criteria for mixed-phenotype acute leukemia, therapy-related myeloid neoplasms, or other AML types with current genetic abnormalities are excluded from this category.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear

Genetics Data

b2a2 fusion
b3a2 fusion
BCR-ABL1 fusion
Chromosome 7 loss
Chromosome 8 gain
p190 fusion
p210 fusion
t(9;22)(q34.1;q11.2)

Immunophenotyping

CD7 aberrant expression
CD13 expression
CD19 aberrant expression
CD33 expression
CD34 expresion
TdT aberrant expression

Treatments

None

Transformations to

None

Corresponding ICD-9 Codes

205.0 Acute myeloid leukemia

Corresponding ICD-10 Codes

C92.0 Acute myeloid leukemia

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

C92.0 Acute myeloblastic leukemia (effective October 01, 2015)

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

CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Physical exam and history
Reverse transcription-polymerase chain reaction test (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

Age: occurs primarily in adults
Incidence: less than 1% of all AMLs
Sex: possible male predominance

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: 140

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