Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1

Name
Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1
ICD-O-3 Morphology
9911/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 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
None
Definition
This is an AML generally showing maturation in the megakaryocyte lineage. This occurs most commonly in infants without Downs Syndrome with a female predominance. This disease is restricted to infants and young children with most cases occurring in the first 6 months of life.
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
RBM15 (aka OTT)
RBM15-MKL1 fusion
t(1;22)(p13;q13)
Immunophenotyping
CD13+
CD33+
CD34-
CD36+
CD41 (glycoprotein IIb/IIIa)
CD42 (glycoprotein Ib-less frequent)
CD45-
CD61 (glycoprotein IIIa)
HLA-DR-
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
Anemia
Easy bruising or bleeding
Elevated white blood cell count
Organomegaly
Shortness of breath
Weakness
Weight loss or loss of appetite
Diagnostic Exams
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