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1. MYELOPROLIFERATIVE NEOPLASM
CHRONIC MYELOID
LEUKAEMIA

2. HISTORY
45 year old present with fatigue and
abdominal distention for the past 6
months. O/E he is pale and his spleen
5cm below coastal margin. His CBC
reveals Hb 10.5g/dl, TLC 120x109
/L and
Platelet count of 550x109
/L.

3. Chronic myelogenous leukaemia
Chronic granulocytic leukaemia
Clonal disorder
Acquired genetic change in
pluripotent haemopoietic stem cell

4. Rare below 20 years of age
Median age of onset is 50 – 60 years
Biphasic or triphasics disease
Intial chronic/indolent or stable
phase which evolves into an advanced
phase

5. Cytogenetics
Translocation of chromosomal
material between long arms of
chromosome 22 and chromosome 9, an
event referred to as t(9;22) (q34:q11)
Generates BCR-ABL fusion gene on
Philadelphia chromosome

7. Ph chromosome

8. Ph chromosome

9. bcr/abl translocation by FISH
Normal nuclei (n)
exhibiting 2 orange
and 2 green signals.
Ph chr. showing
Yellow fusion signal of
orange and green
signals ( arrows

10. bcr/abl translocation by FISH

11. Presentation
Abdominal discomfort or
progressive enlargement
Weight loss
Lethargy
Loss of energy
Shortness of breath
Visual disturbances

12. Presentations
Spontaneous bruising
Bleeding gums
Infections

13. Diagnosis
CBC
Anaemia
Raised TLC
DLC
Full spectrum of granulocytic
cells ( Blast, myelocyte,
metamyelocyte, band cell,
Neutrophils)

14. Eosinophils
Basophils
Platelet count raised
Bone marrow examination:
Degree of fibrosis
Cytogenetic analysis
Exclude incipient
transformation

15. Raise LDH
Reduced LAP score
Raised vit B12 levels
Hypercalcaemia
Raised serum uric acid

16. Phases
Chronic Phase:
Ability to reduce spleen size and
restore and maintain a normal blood
count with therapy
Blast cell percentage is less than 10%
Eosinophils and basophils are
normal in number

17. Accelerated Phase:
Blast 10 – 19%
Persistent thrombocytopenia unrelated to
therapy or thrombocytosis unresponsive
to therapy
Increasing spleen size and counts
unresponsive to therapy
Megakaryocyte proliferation and reticluin
or collagen fibrosis

18. Blastic Phase:
Blast >20%
Extramedullary blast proliferation