At which age is the peak incidence?
peak incidence is 50–60 years
Describe the causes.
Idiopathic (in most cases)
Ionizing radiation (e.g., secondary to therapeutic radiation)
Aromatic hydrocarbons (especially benzene)
Describe the pathophysiology.
Reciprocal translocation between chromosome 9 and chromosome 22 → formation of the Philadelphia chromosome t(9;22) → fusion of the ABL1 gene (chromosome 9) with the BCR gene (chromosome 22) → formation of the BCR-ABL gene → encodes a BCR-ABL non-receptor tyrosine kinase with increased enzyme activity
Result: inhibits physiologic apoptosis and increases mitotic rate → uncontrolled proliferation of functional granulocytes
Detection of philadelphia translocation (table).
Describe genetic changes and clinical course.
Additional chromosomal changes and mutations of tumor suppressor genes and oncogenes (p53, Rb1, or Ras), which emerge during the course of the disease, are responsible for the progression from chronic to accelerated phase and, ultimately, the transition to acute leukemia.
Last changed2 years ago
