9875/3 Chronic myelogenous leukemia, Philadelphia chromosome (Ph 1) positive



Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C92.1
           

/span>hronic myelogenous leukaemia (CML) is a myeloproliferative neoplasm that originates in an abnormal pluripotent bone marrow (BM) stem cell and is consistently associated with the BCR-ABL1 fusion gene located in the Philadelphia (Ph) chromosome 1
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Bartram CR, de Klein A, Hagemeijer A, van Agthoven T, Geurts van Kessel A, Bootsma D, Grosveld G, Ferguson-Smith MA, Davies T, Stone M (1983)
Translocation of c-ab1 oncogene correlates with the presence of a Philadelphia chromosome in chronic myelocytic leukaemia.
Nature 306: 277-80



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Melo JV, Barnes DJ (2007)
Chronic myeloid leukaemia as a model of disease evolution in human cancer.
Nat Rev Cancer 7: 441-53



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Rowley JD (1973)
Letter: A new consistent chromosomal abnormality in chronic myelogenous leukaemia identified by quinacrine fluorescence and Giemsa staining.
Nature 243: 290-3



. Although the initial major finding is neutrophilic leukocytosis, the BCR-ABL1 is found in all myeloid lineages as well as in some lymphoid cells and endothelial cells. The natural history of untreated CML is bi- or triphasic: an initial indolent chronic phase (CP) is followed by an accelerated phase (AP), a blast phase (BP) or both.

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