Coding Manual:
Hematopoietic Coding Manual (PDF)
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.
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.
Diagnostic Confirmation
This AML is part of the "AML with recurrent genetic abnormalities" group. Since this AML is diagnosed based on genetics, diagnostic confirmation will always be 3.
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/positive)
CD13+ (expression/positive)
CD19+ (aberrant expression/positive)
CD33+ (expression/positive)
CD34+ (expression/positive)
TdT+ (aberrant expression/positive)
Treatments
None
Transformations to
None
Transformations from
Same Primaries
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
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
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
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq