Chronic myelogenous leukemia is a bone marrow cancer driven by the BCR-ABL1 fusion gene on the Philadelphia chromosome, which makes too many mature-looking granulocytes. Most patients are diagnosed in the chronic phase, often from a routine blood count, and oral targeted drugs give a near normal life expectancy.
Key symptoms
- No symptoms in many; a high white count on a routine test
- Fatigue, weight loss and night sweats
- Fullness in the upper left belly or feeling full quickly
- Bone pain, bleeding or fever in advanced phases
What you find on exam
- Enlarged spleen, often large
- Pallor
- Enlarged liver in some patients
- Signs of leukostasis: retinal bleeding or neurologic changes
Tests
- Blood count with smear: high white count with all stages of granulocytes, more basophils, often high platelets
- BCR-ABL1 by quantitative PCR and FISH
- Bone marrow biopsy with cytogenetics to define the phase
- Chemistry with uric acid and LDH
Treatment basics
- Tyrosine kinase inhibitor first line: imatinib, dasatinib, nilotinib or bosutinib
- Monitor BCR-ABL1 by PCR every 3 months to track response
- Poor response: check adherence and mutations, then switch drugs; asciminib for resistance
- Stem cell transplant for blast phase or failure of several drugs
- Some patients in deep, lasting remission can try stopping under close monitoring
Red flags
- Blast crisis: rising blasts, fever, bleeding and bone pain
- Very high white count with headache, vision changes or breathlessness (leukostasis)
- Sudden severe upper left belly pain (splenic infarct or rupture)
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Sources: NCCPA PANCE Content Blueprint; NBOME COMLEX-USA clinical presentations; AAMC Core Entrustable Professional Activities (Core EPAs). Written by Making Synapses. Making Synapses is not affiliated with these organizations.