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

Name
Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11
ICD-O-3 Morphology
9871/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 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, 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
Acute myelomonocytic leukemia with abnormal eosinophils
FAB M4Eo
Definition
Acute myeloid leukemia that usually shows monocytic and granulocytic differentiation and the presence of a characteristically abnormal eosinophil component in the marrow. Myeloid sarcomas may be present at initial diagnosis or relapse
Abstractor Notes
The acute myeloid leukemias with related precursor neoplasms are characterized by recurrent genetic abnormalities of prognostic significance. This specific disease 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 of disease.

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
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 (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
Complete blood count (CBC)
CT (CAT) scan
Cytogenetic analysis
Reverse transcription-polymerase chain reaction test (RT-PCR)
Recurrence and Metastases
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