Name

Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11

ICD-O-3 Morphology

9871/3: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16) (p13.1;q22), CBFB/MYH11
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

See abstractor notes

Alternate Names

Acute myeloid leukemia, CBF-beta/MYH11
Acute myeloid leukemia, inv(16)(p13;q22)
Acute myeloid leukemia, t(16;16)(p13;q11)
Acute myeloid leukemia with abnormal marrow eosinophils (includes all variants)
Acute myelomonocytic leukemia with abnormal eosinophils
FAB M4Eo

Definition

Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22) resulting in CBFB-MYH11 is an AML that usually shows monocytic and granulocytic differentiation and characteristically an abnormal eosinophil component in the bone marrow.

Abstractor Notes

The acute myeloid leukemias with related precursor neoplasms are characterized by recurrent genetic abnormalities of prognostic significance. This specific neoplasm is an acute myeloid leukemia with abnormal marrow eosinophils.

In addition to the usual morphological features of acute myelomonocytic leukemia, the BM shows a variable number of eosinophils (usually increased but sometimes less than five percent). The most striking abnormalities include the immature eosinophilic granules, usually present at later stages.

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

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

CBFB at 16q22 to MYH11 at 16p13.1 fusion
inv(16)(p13.1q22)
t(16;16)(p13.1;q22)

Immunophenotyping

CD4+ monocytic
CD11b+ monocytic
CD11c+ monocytic
CD13+ granulocytic
CD14+ monocytic
CD15+ granulocytic
CD33+ granulocytic
CD36+ monocytic
CD64+ monocytic
CD65+ granulocytic
Lysozyme+ monocytic
MPO+ granulocytic

Treatments

Chemotherapy

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

Easy bruising or bleeding
Shortness of breath
Weakness
Weight loss or loss of appetite

Progression and Transformation

Longer complete remissions when treated with high dose cytarabine in the consolidation phase
Older patients and those with KIT mutations have higher risk of relapse and worse survival

Epidemiology and Mortality

Age: predominantly younger patients
Incidence: 5-8% of all cases of AML
Survival: longer complete remissions possible when treated with high dose cytarabine in the consolidation phase

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: 132-134

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

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