Chronic myeloid leukemia (CML), also known as chronic myelogenous leukemia, is a myeloproliferative disorder defined by a unique molecular / cytogenetic abnormality in which a balanced translocation between chromosomes 9 and 22 occurs to create the Philadelphia (Ph) chromosome [t(9;22)(q34;q11.2)]. The novel BCR-ABL1 fusion oncogene codes for a protein with tyrosine kinase activity that is constitutively active, leading to activation of many downstream pathways. The result is excessive proliferation and reduced apoptosis of CML cells, causing reduced hematopoiesis.
CML represents about 15%-20% of all leukemias in the United States. Median age at diagnosis is between 50 and 65 years, and there is a slight male predominance.
CML begins in the chronic phase (CP); this may progress to an accelerated phase (AP) and, later, a blast phase (BP), or acute leukemia (> 20% blasts). Approximately 85% of patients will be in CP when they present or are diagnosed.
Related topic: acute myelogenous leukemia
Chronic myeloid leukemia in Adult
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Synopsis
Codes
ICD10CM:
C92.10 – Chronic myeloid leukemia, BCR/ABL-positive, not having achieved remission
SNOMEDCT:
92818009 – Chronic myeloid leukemia, disease
C92.10 – Chronic myeloid leukemia, BCR/ABL-positive, not having achieved remission
SNOMEDCT:
92818009 – Chronic myeloid leukemia, disease
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Leukemoid reaction – reactive nonclonal proliferation of WBC due to infection or stress
- Chronic lymphoid leukemia – increased WBC; however, differential shows they are lymphocytes
- Chronic neutrophilic leukemia – elevated WBC composed of predominantly neutrophils (80%) along with circulating blasts may be associated with mutation in CSF3R, which is a growth factor receptor
- Acute myeloid leukemia – increased WBC made up of > 20% myeloblasts
- Acute lymphoid leukemia – increased WBC made up of > 20% lymphoblasts
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:06/10/2019
Last Updated:09/13/2023
Last Updated:09/13/2023
Chronic myeloid leukemia in Adult