Name

Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3

ICD-O-1 Morphology

9897/3: Chronic monocytic leukemia
Effective 1978 - 1991

ICD-O-2 Morphology

9897/3: Chronic monocytic leukemia
Effective 1992 - 2000

ICD-O-3 Morphology

9897/3: Acute myeloid leukemia with t(9;11)(p22;q23);MLLT3-MLL
Effective 2001 and later

Reportable

for cases diagnosed 1978 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, MLL
Acute myeloid leukemia with 11q23 (MLL) abnormalities
Acute myeloid leukemia with t(9;11)(p22;q23); MLLT3-MLL
Acute myeloid leukemia with t(9;11)(p22;q23) resulting in KMT2A-MLLT3

Definition

Acute myeloid leukemia with t(9;11)(p21.3;q23.3) resulting in KMT2A-MLLT3 fusion is usually associated with monocytic features.

Abstractor Notes

The provisional diagnoses may include
1. AML
2. AML without maturation
3. Acute lymphoblastic leukemia (ALL)
4. AML with maturation.

The patients with ALL have a low percentage of MPO-positive blasts while the AML with maturation has a higher percentage of MPO-positive blasts.

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

For more information on acute myeloid leukemia, see the NCI website: http://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq or http://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

KMT2A (previously called MLL and HRX)
MECOM (also called EV11) overexpression
t(9;11)(p21.3;q23.3) involving MLLT3
t(9;11)(p21.3;q23.3) with gain of chromosome 8

Immunophenotyping

CD4 strong expression and monocytic differentiation
CD11b monocytic differentiation
CD11c monocytic differentiation
CD13 low expression
CD14 low expression and monocytic differentiation
CD33 strong expression
CD34 low expression
CD36 monocytic differentiation
CD56 variable expression
CD64 monocytic differentiation
CD65 strong expression
HLA-DR strong expression
KIT (CD117) variable expression
Lysozyme variable expression & monocytic differentiation
NG2 homologue expression encoded by CSPG4

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)

C92.6 Acute myeloid leukemia with 11q23-abnormality (effective October 01, 2015)

Signs and Symptoms

Dissminated intravascular coagulation
Easy bruising or bleeding
Shortness of breath
Tissue infiltration (gingiva, skin)
Weakness
Weight loss or loss of appetite

Progression and Transformation

None

Epidemiology and Mortality

Age: more common in children, occurs also in adults
Incidence: 9-12% of childhood and 2% of adult AML cases
Survival: intermediate survival

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: 136-137

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