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

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
M3 Module 5: PH10
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 AML, AML without maturation, ALL, and 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. The patients usually present with BM failure with anemia, thrombocytopenia, and neutropenia.
Definitive Diagnostic Methods
Bone Marrow biopsy
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
No Transformations
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None