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)

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.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9910/3.)

Most commonly occurs in infants without Downs syndrome.

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5: PH10

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

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

Easy bruising or bleeding
Elevated white blood cell count
Organomegaly
Shortness of breath
Weakness
Weight loss or loss of appetite

Progression and Transformation

Extramedullary disease or myeloid sarcoma indicative of more definitive AML

Epidemiology and Mortality

Age: Infants without Down syndrome
Incidence: <1% of AML cases
Sex: female predominance
Survival: poor to good response depending on treatment

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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013. (http://codes.iarc.fr/codegroup/2)
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary