Acute myeloid leukemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1-EVI1

Name
Acute myeloid leukemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1-EVI1
ICD-O-3 Morphology
9869/3
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
None
Alternate Names
None
Definition
This is an AML that may present de novo or arise from a prior MDS. It is often associated with normal or elevated peripheral blood platelet counts and has increased atypical bone marrow megakaryocytes with mono- or bi-lobated nuclei and associated multilineage dysplasia.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9861/3.)

Patients may have a prior MDS or present with splenomegaly.

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5:PH10
Definitive Diagnostic Methods
Genetic testing
Immunophenotyping
Peripheral blood smear
Genetics Data
EVl1
MDS1-EV11
RPN1
Immunophenotyping
CD13
CD33
CD34
CD38
HLA-DR
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
Shortness of breath
Weakness
Weight loss or loss of appetite
Diagnostic Exams
Progression and Transformation
None
Epidemiology and Mortality
Age: predominantly adults
Incidence: 1-2% of all AML cases
Sex: no male or female predominance
Survival: aggressive disease with short survival