Synonyms
Definition
Chronic myeloid leukemia (CML) represents the clonal and neoplastic proliferation of a hematopoietic stem cell with a chromosomal translocation t(9;22) and junction of the BCR-gene and ABL-gene resulting in a BCR-ABL fusion protein of 210 kD or 190 kD, and leading to growth factor independent proliferation and leucocytosis. Non-neoplastic stem cells are vergrown but differentiation and maturation are retained by neoplastic cells with sufficient production of blood cells. In untreated patients, the steady state phase (chronic phase) with sufficient production of mature blood cells usually progresses to an accelerated phase with deterioration of hematologic blood parameters and/or organomegaly. Differentiation of the transformed hemaopoietic stem cells gets lost after a period of about 3 years (median) resulting in the blast phase. Transition from chronic to accelerated and blast phase, respectively, is defined by a number of parameters,...
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References and Further Reading
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Kreipe, H. (2019). Chronic Myeloid Leukemia, BCR-ABL1 Positive. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5013-1
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