Chronic Myeloid Leukaemia

Question 1

Define:

Answer 1

Malignant clonal disease characterised by uncontrolled proliferation of myeloid cells in the bone marrow and blood.

Slower progression than AML

Question 2

Aetiology:

Answer 2

Malignant proliferation of stem cells - 95% have the Philadelphia chromosome (translocation of 9 and 22 BCR-ABL fusion gene)

Other types:

• Ph -ve (worse prognosis)
• Chronic neutrophilic/eosonphilic

Question 3

What are the stages of CML:

Answer 3

o Relatively stable chronic phase (4-6 yr duration)
o Accelerated phase (3-9 months)
o Acute leukaemia phase - blast transformation

Question 4

Risk factors:

Answer 4

Ionising radiation
Benzene
IBD

Question 5

Epidemiology:

Answer 5

Incidence increases with age
4x more in males
peak incidence in 40-60 years

Question 6

Symptoms:

Answer 6

Asymp in 40-50%
Weight loss
Malaise
Sweating

Bone marrow failure:

• lethargy
• dyspnoea
• Easy bruising
• Epitaxisis
• Abdo discomfort and early satiety due to the splenic enlargement

rare - gout and hypervicosity (headaches, visual disturbnces and priapism)

Question 7

Signs:

Answer 7

Splenomegaly - 90% of cases and the most common finding
Pallor
Bleeding
Ecchymosis

Question 8

Investigations:

Answer 8

o	FBC 
•	High WCC with whole spectrum of myeloid cells – high neutrophils, myelocytes, basophils, eosinophils
•	Low Hb 
•	High/normal/low platelets 
•	High uric acid 
•	High B12 and transcobalamin I 
•	Low neutrophil ALP score 

Blood Film
o Immature granulocytes

Bone Marrow Aspirate or Biopsy
o Hypercellular with raised myeloid-erythroid ratio

Cytogenetics
o Show the Philadelphia chromosome