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

Help me code for dx year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
aCML
Atypical chronic myeloid leukemia
Philadelphia chromosome (PH1) negative
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. Although most patients are in their 70s or 80s, there have been cases reported in teenagers. The peripheral blood and bone marrow are always involved; spleen and hepatic involvement are common. The chief complaint of patients is splenomegaly. Most patients have symptoms related to anemia or occasionally thrombocytopenia. The physician will run tests to rule out BCR-ABL1 involvement. Patients with aCML fare poorly. The series reported to the present time include only small numbers of patients but median survival times range from 14-29 months.
Definitive Diagnostic Methods
Bone Marrow Biopsy
Peripheral blood smear
Genetics Data
None
Immunophenotyping
None
Treatments
Chemotherapy
Immunotherapy
Stem cell transplant
Transformations to
No Transformations
Transformations from
No 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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None