Name

Acute myeloid leukemia with myelodysplasia-related changes

ICD-O-3 Morphology

9895/3: Acute myeloid leukemia with myelodysplasia-related changes
Effective 2001 and later

Reportable

for cases diagnosed 2001 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 with multilineage dysplasia
Acute myeloid leukemia with prior myelodysplastic syndrome
Acute myeloid leukemia without prior myelodysplastic syndrome
AML-MRC

Definition

Acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) is an acute leukemia with greater than or equal to 20% peripheral blood or bone marrow blasts with morphological features of myelodysplasia, or occurring in patients with a prior history of a myelodysplastic syndrome (MDS) or myelodysplastic/myeloproliferative neoplasms (MDS/MPN), with MDS-related cytogenetic abnormalities; the specific genetic abnormalities characteristic of AML with recurrent genetic abnormalities are absent.

Abstractor Notes

These patients DO NOT have a history of prior cytotoxic or radiation therapy for an unrelated disease. The provisional diagnoses during work-up may include refractory anemia with excess blasts; acute erythroid leukemia, acute megakaryoblastic leukemia and AML, NOS.

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
Peripheral blood smear

Genetics Data

CEBPA
del (5q)
del(7q)
FLT3
NPM1
t(2;11)(p21;q23.3)
t(11;16)(q23.3;p13.3)
5q32-33
11q23.3 rearrangements

Immunophenotyping

CD7 aberrant expression
CD11b expression
CD13 expression
CD14 expression
CD33 expression
CD34 expression
CD38 low expression
CD56 aberrant expression
FLT3 (CD135) decreased expression
HLA-DR decreased expression
KIT (CD117) decreased expression
TdT expression

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.A Acute myeloid leukemia with multilineage dysplasia (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Fatigue
Fever
Petechiae
Severe pancytopenia
Shortness of breath
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

Lower rate of achieving complete remission than other AML types

Epidemiology and Mortality

Age: elderly, rare in children
Incidence: 24-35% of all AML cases (variable based on definition)
Survival: generally poor, lower rate of achieving remission than other types of AML

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: 150-152

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
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