C92.20

HCC Category Mapping

What This Code Means

A rare type of myeloid leukemia without the typical genetic mutation, where the patient has not achieved remission.

Coding Tips

• •Confirm BCR/ABL-negative status is documented to distinguish from typical chronic myeloid leukemia
• •Document that remission has not been achieved; this indicates active disease

Clinical Significance

Atypical chronic myeloid leukemia (aCML), BCR/ABL-negative, is a rare myelodysplastic/myeloproliferative neoplasm that shares clinical features with CML but lacks the Philadelphia chromosome and BCR-ABL1 fusion gene. aCML typically presents with leukocytosis, multilineage dysplasia, and a worse prognosis than classic CML. The 'not in remission' status indicates ongoing active disease that is not responding to treatment.

Documentation Requirements

• ✓Documentation must confirm BCR/ABL-negative status by cytogenetics, FISH, or molecular testing.
• ✓The diagnosis requires granulocytic dysplasia, leukocytosis with immature granulocyte precursors, and absence of BCR-ABL1, PDGFRA, PDGFRB, or FGFR1 rearrangements.
• ✓Molecular testing for SETBP1 and ETNK1 mutations (common in aCML) should be documented when performed.
• ✓Active disease status and treatment response must be recorded.

Commonly Confused Codes