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Chronic myeloid leukaemia
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Chronic myeloid leukaemia

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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
  • 6. Ph chromosome
  • 7. Ph chromosome
  • 8. 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
  • 9. bcr/abl translocation by FISH
  • 10. Presentation Abdominal discomfort or progressive enlargement Weight loss Lethargy Loss of energy Shortness of breath Visual disturbances
  • 11. Presentations Spontaneous bruising Bleeding gums Infections
  • 12. Diagnosis CBC Anaemia Raised TLC DLC Full spectrum of granulocytic cells ( Blast, myelocyte, metamyelocyte, band cell, Neutrophils)
  • 13. Eosinophils Basophils Platelet count raised Bone marrow examination: Degree of fibrosis Cytogenetic analysis Exclude incipient transformation
  • 14. Raise LDH Reduced LAP score Raised vit B12 levels Hypercalcaemia Raised serum uric acid
  • 15. 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
  • 16. 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
  • 17. Blastic Phase: Blast >20% Extramedullary blast proliferation