Natural history

• Biphasic or triphasic disease—chronic phase, accelerated phase and blast crisis; 50% transform directly from chronic phase to blast crisis.

• >85% patients present in chronic phase.

• Duration of chronic phase varies (typically 3-6 years; median 4.2 years).

• Transformation is least likely in the 2 years immediately after diagnosis but occurs at an annual rate of 20-25% thereafter.

• Accelerated phase characterised by blood counts and organomegaly becoming increasingly refractory to therapy; some have constitutional symptoms; generally brief.

• Blast crisis resembles acute leukaemia with >20% blasts and promyelocytes in blood or marrow.

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