Acute myeloid leukemia with t(9;11)(p22;q23); MLLT3-MLL

Name
Acute myeloid leukemia with t(9;11)(p22;q23); MLLT3-MLL
ICD-O-1 Morphology
9897/3
Effective 1978 - 1991
ICD-O-2 Morphology
9897/3
Effective 1992 - 2000
ICD-O-3 Morphology
9897/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

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Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
See abstractor notes
Alternate Names
Acute myeloid leukemia with 11q23 (MLL) abnormalities
Acute myeloid leukemia, 11q23 abnormalities
Acute myeloid leukemia, MLL
Definition
Note: 11q23 abnormalities don't always correlate with MLL gene rearrangement

AML with 11q23 abnormalities is usually associated with monocytic features. More common in children. Two subgroups of patients exhibit 11q23: 1) AML in infants and 2) therapy-related leukemia usually occurring after treatment with DNA topoisomerase II inhibitors

Patients may present with DIC and extramedullary monocytic sarcomas and/or tissue infiltration (gingiva, skin).

Translocations involving chromosome band 11q23 occur frequently in both AML and ALL; at least 30 different partner chromosomes are involved in recurring reciprocal 11q23 translocations, suggesting that the 11q23 breakpoint region may contain genomically unstable sequences that lead to chromosomal recombination events.
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.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
11q23
MLL (aka HRX)
t(6;11)(q27;q23)
t(9;11)(p21;q23)
t(9;11)(p22;q23)
t(11;19)(q23;p13.1)
t(11;19)(q23;p13.3)
Immunophenotyping
CD4 (strong expression & monocytic differentiation)
CD11b (monocytic differentiation)
CD11c (monocytic differentiation)
CD13 (low expression)
CD14 (low expression & monocytic differentiation)
CD33 (strong expression)
CD34 (low/variable expression)
CD36 (monocytic differentiation)
CD56 (variable expression)
CD64 (monocytic differentiation)
CD65 (strong expression)
CD117 (variable expression)
HLA-DR (strong expression)
Lysozyme (variable expression & monocytic differentiation)
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 (effective October 1, 2015 U.S. only)
C92.6 Acute myeloid leukemia with 11q23-abnormality
Signs and Symptoms
Anemia
Dissminated intravascular coagulation
Easy bruising or bleeding
Shortness of breath
Tissue infiltration (gingiva, skin)
Weakness
Weight loss or loss of appetite
Diagnostic Exams
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