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

Name
Acute myeloid leukemia with t(6;9)(p23;q34); DEK-NUP214
ICD-O-3 Morphology
9865/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
See abstractor notes
Alternate Names
None
Definition
This disease is an AML with or without monocytic features that is often associated with basophilia and multilineage dysplasia. Patients usually present with anemia and thrombocytopenia. White blood cell count is generally lower than other AML types
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 disease 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
DEK
NUP214
Immunophenotyping
CD13
CD15
CD33
CD34
CD38
CD117
HLA-DR
Myeloperoxidase
Treatments
Stem cell transplant
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
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