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Chronic Myeloid Leukemia (CML)

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Concise Guide to Hematology

Abstract

Chronic myeloid leukemia (CML) is a malignant disease of hematopoietic progenitor cells. It arises from a t(9;22) which is a reciprocal chromosomal translocation between the ABL proto-oncogene and the BCR gene on chromosome 22. The resulting product (BCR-ABL1) is a constitutively active tyrosine kinase leading to uncontrolled proliferation of myeloid, monocytic, erythroid, megakaryocytic, B-lymphoid, and occasionally T-lymphoid cell lineages. CML can be classified as being in chronic, accelerated, or blastic phase which impacts both treatment and prognosis. For patients in chronic phase, once the diagnosis is confirmed, therapy with a tyrosine kinase inhibitor (TKI) should be started. Patients should then be monitored serially with a hematologist following ELN criteria. If patients progress or lose their response, adherence to therapy should be investigated and a BCR-ABL1 mutational panel sent to guide the next step in therapy. For those that remain in remission, there is emerging data that TKI therapy can be safely discontinued in a subset of patients who have achieved deep remission and maintained it for several years. For patients presenting in accelerated phase and blast crisis, treatment remains challenging even in the age of TKIs. Generally, patients are first given induction therapy followed by allogeneic hematopoietic cell transplantation (HCT), although, in the era of increasing TKI options, there has not been a randomized trial looking at HCT versus continuing to the next TKI if response is lost.

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Authors and Affiliations

  1. Levine Cancer Institute, Atrium Health, Charlotte, NC, USA

    Thomas G. Knight, Michael R. Grunwald & Edward A. Copelan

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  1. Thomas G. Knight
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  2. Michael R. Grunwald
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  3. Edward A. Copelan
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Correspondence to Thomas G. Knight .

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Editors and Affiliations

  1. Department of Medicine, Case Western Reserve University, Cleveland, OH, USA

    Hillard M. Lazarus

  2. Department of Medicine, University Hospital Cleveland Medical Center, Cleveland, OH, USA

    Alvin H. Schmaier

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Knight, T.G., Grunwald, M.R., Copelan, E.A. (2019). Chronic Myeloid Leukemia (CML). In: Lazarus, H., Schmaier, A. (eds) Concise Guide to Hematology. Springer, Cham. https://doi.org/10.1007/978-3-319-97873-4_25

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  • DOI: https://doi.org/10.1007/978-3-319-97873-4_25

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