Haematology & Oncology

Chronic Myeloid Leukaemia (CML)

A single acquired fusion gene, BCR-ABL1 from the Philadelphia chromosome, creates a constitutively active tyrosine kinase that drives myeloid overproduction with preserved maturation, so the blood fills with maturing granulocytes and the spleen enlarges, usually indolently until it is controlled by a targeted inhibitor.

Definition

Chronic myeloid leukaemia is a myeloid neoplasm defined by the BCR::ABL1 fusion, usually produced by the Philadelphia chromosome. Chronic phase retains granulocyte maturation and is managed with long-term molecularly monitored TKI therapy.

First principles

One fusion kinase drives the whole disease

CML is driven by the Philadelphia chromosome rearrangement that creates the BCR::ABL1 fusion tyrosine kinase. This explains the disease biology and the effectiveness of BCR::ABL1-directed TKI therapy.

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