Demographics:
- rare disease, 0.03% of all cancers diagnosed.
- CML can occur at any age, more common >40yo
- men>women
- rarely diagnosed in children.
Aetiology:
- Idiopathic (in most cases)
- Ionizing radiation (e.g., secondary to therapeutic radiation)
- Aromatic hydrocarbons (especially benzene)
Risk factors
- Ionizing radiation (e.g., secondary to therapeutic radiation)
- Aromatic hydrocarbons (especially benzene)
Pathophysiology
- translocation (9:22) → fusion/activation of BCR-ABL oncogene → encodes non-receptor tyrosine kinase → inhibits physiologic apoptosis and increases mitotic rate → uncontrolled proliferation of granulocytes (increased mature cells, mid stage progenitor cells (myelocytes, metamyelocytes) but no blasts)
3 phases