Acute myeloid leukemia with t(8;21)(q22;q22); RUNX1-RUNX1T1

Name
Acute myeloid leukemia with t(8;21)(q22;q22); RUNX1-RUNX1T1
ICD-O-3 Morphology
9896/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
See abstractor notes
Alternate Names
Acute myeloid leukemia, AML1(CBF-Alpha)/ETO
Acute myeloid leukemia, FLT3-TKD
Acute myeloid leukemia, t(8;21)(q22;q22)
Acute myeloid leukemia with recurrent genetic abnormalities
AML1/ETO
FAB M2, AML1(CBF-Alpha)/ETO
FAB M2, t(8;21)(q22;q22)
Definition
The t(8;21)(q22;q22) translocation juxtaposes the AML1 gene on chromosome 21 to the ETO for t(I;21) gene on chromosome 8, which results in the production of the AML-ETO fusion protein.
AML with t(8;21)(q22;q22) is an acute myeloid leukemia generally showing maturation in the neutrophil lineage. This translocation is one of the most common (5-12% of AML cases).
Myeloid sarcomas (chloromas) may be present and may be associated with a bone marrow blast percentage of less than 20%.
If translocation present, categorize as AML even if initial blast count is < 20%.
Abstractor Notes
Tumor manifestations such as myeloid sarcomas may be present at diagnosis; in these cases an initial BM aspiration may show a misleading low number of blast cells but should be diagnosed as AML despite a blast percentage in BM of less than 20. Smaller blasts may be found in the PB.

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
del(9q)
RUNX1-RUNX1T1
t(8;21)(q22;q22)
Immunophenotyping
CD13
CD33 (weak expression)
CD34
CD56 (expressed in a fraction of cases)
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)
C92.0 Acute myeloblastic leukemia
Signs and Symptoms
Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite
Diagnostic Exams
Bone marrow aspiration and biopsy
Complete blood count (CBC)
CT (CAT) scan
Reverse transcription-polymerase chain reaction test (RT-PCR)
Progression and Transformation
High complete remission rate with long term disease-free survival when treated with high dose cytarabine in the consolidation phase
Epidemiology and Mortality
Age: predominantly younger patients
Incidence: ~5-10% of AML cases
Survival: High complete remission with possible long term disease free survival when treated with high dose cytarabine