Name

Acute myeloid leukemia with t(6;9)(p23;q34.1); DEK-NUP214

ICD-O-3 Morphology

9865/3: Acute myeloid leukemia with t(6;9)(p23;q34);DEK-NUP214
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 with t(6;9)(p23;q34); DEK-NUP214

Definition

Acute myeloid leukemia with t(6;9)(p23;q34.1) resulting in DEK-NUP214 is an AML with greater than or equal to 20% peripheral blood or bone marrow blasts with or without monocytic features. It is often associated with basophilia and multilineage dysplasia.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9861/3.)

This AML has a generally poor prognosis. Elevated white blood cell counts are most predictive of shorter overall survival and increased BM blasts are associated with shorter free survival. Very limited data available regarding treatment or survival.

In adults the WBC is generally lower than other AML types. The BM may have morphologic and cytochemical features of any FAB subtype of AML other than acute promyelocytic leukemia and acute megakaryoblastic leukemia.

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

FLT3-ITD
NUP214 (also called CAN) on chromosome 9

Immunophenotyping

CD9 expression
CD13 expression
CD15 expression
CD33 expression and positive
CD34 expression
CD38 expression and positive
CD64 expression
CD123 positive
HLA-DR negative
KIT (CD117)
MPO expression

Treatments

Hematologic Transplant and/or Endocrine Procedures

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
Pancytopenia
Shortness of breath
Weakness
Weight loss or loss of appetite

Progression and Transformation

Elevated white blood cell counts associated with shorter overall survival
Increased bone marrow blasts associated shorter disease-free survival

Epidemiology and Mortality

Age: 13 years median age in children, 35 years median age in adults
Incidence: 0.7-1.8% of AML cases
Survival: poor prognosis for both children and adults

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: 137-138

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