
Name
Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1
ICD-O-3 Morphology
9911/3: Acute myeloid leukamia (megakaryoblastic) with t(1;22)(p13;q13);RBM15-MKL1
Effective
2010 and later
Reportable
for cases diagnosed
2010 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Diagnostic Confirmation
This AML is part of the "AML with recurrent genetic abnormalities" group. Since this AML is diagnosed based on genetics, diagnostic confirmation will always be 3.
Grade
Not Applicable
Module Rule
See abstractor notes
Alternate Names
Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1
Definition
Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1) resulting in RBM15-MKL1fusion is an AML generally showing maturation in the megakaryocyte lineage.
Definitive Diagnostic Methods
Bone marrow biopsy
Cytogenetics
Genetic testing
Immunophenotyping
Peripheral blood smear
Genetics Data
MKL1 (also called MAL)
RBM15 (also called OTT)
RBM15-MKL1 fusion
t(1;22)(p13.3;q13.1)
Immunophenotyping
CD13 positive
CD33 positive
CD34 negative
CD36 positive, but not specific
CD41 (glycoprotein IIb/IIIa) expression
CD42b (glycoprotein Ib) expression
CD45 negative
CD61 (glycoprotein IIIa) expression
HLA-DR negative
MPO negative antibodies
TdT not expressed
Treatments
Chemotherapy
Transformations to
None
Transformations from
Same Primaries
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)
C92.0 Acute myeloblastic leukemia (effective October 01, 2015)
Signs and Symptoms
Anemia
Easy bruising or bleeding
Elevated white blood cell count
Fatigue
Fever
Hepatosplenomegaly
Organomegaly
Petechiae
Shortness of breath
Splenomegaly
Thrombocytopenia
Weakness
Weight loss or loss of appetite
Diagnostic Exams
CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Physical exam and history
Reverse transcription-polymerase chain reaction test (RT-PCR)
Progression and Transformation
Extramedullary disease or myeloid sarcoma indicative of more definitive AML
Epidemiology and Mortality
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: 139-140
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 139-140
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
