Atypical chronic myeloid leukemia, BCR-ABL1 negative

Name
Atypical chronic myeloid leukemia, BCR-ABL1 negative
ICD-O-3 Morphology
9876/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
aCML
Definition
aCML is a leukemic disorder with myelodysplastic as well as myeloproliferative features. It is characterized by principal involvement of the neutrophil series with leukocytosis comprised of immature and mature neutrophils that are dysplastic. Mutilineage dysplasia; however, is common and reflects the stem cell origin.
Abstractor Notes
Atypical CML BCR-ABL1 negative is a rare disease. The peripheral blood and bone marrow are always involved; spleen and hepatic involvement are common.

The physician will run tests to rule out BCR-ABL1 involvement.
Definitive Diagnostic Methods
Bone marrow biopsy
Peripheral blood smear
Genetics Data
None
Immunophenotyping
None
Treatments
Chemotherapy
Immunotherapy
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
205.1 Chronic myeloid leukemia
Corresponding ICD-10 Codes
C92.1 Chronic myeloid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C92.2 Atypical chronic myeloid leukemia, BCR/ABL-negative
Signs and Symptoms
Anemia
Easy bruising or bleeding
Frequent infections
Pain or a feeling of fullness below the ribs
Pale skin
Shortness of breath
Weakness
Progression and Transformation
15-40% of patients evolve to acute myeloid leukemia
Epidemiology and Mortality
Age: 70-80 years median age
Incidence: 1-2 cases for every 100 cases of BCR-ABL1 positive (9875/3)
Sex: no male or female predominance
Survival: 14-29 months median survival time