Abstract
Chronic myelogenous leukemia (CML) is a malignancy of the myeloid cell lineage characterized by a recurrent chromosomal abnormality: the Philadelphia chromosome, which results from the reciprocal translocation of the chromosomes 9 and 22. The Philadelphia chromosome contains a fusion gene called BCR-ABL1. The BCR-ABL1 codes for an aberrantly functioning tyrosine kinase that drives the malignant proliferation of the founding clone. The advent of tyrosine kinase inhibitors (TKI) represents a landmark in the treatment of CML, that has led to tremendous improvement in the remission and survival rates. Since the introduction of imatinib, the first TKI, several other TKI have been approved that further broadened the arsenal against CML. Patients treated with TKIs require sensitive monitoring of BCR-ABL1 transcripts with quantitative real-time polymerase chain reaction (qRT-PCT), which has become an essential part of managing patients with CML. In this review, we discuss the importance of the BCR-ABL1 assay, and we highlight the growing importance of BCR-ABL1 dynamics. We also introduce a mathematical correction for the BCR-ABL1 assay that could help homogenizing the use of the ABL1 as a control gene. Finally, we discuss the growing body of evidence concerning treatment-free remission. Along with the continuous improvement in the therapeutic arsenal against CML, the molecular monitoring of CML represents the avant-garde in the struggle to make CML a curable disease.
Highlights
Chronic myelogenous leukemia (CML) is a malignant proliferation driven by a characteristic fusion gene called BCR-ABL1 [1]
Cytogenetic studies, CML is further subdivided into chronic phase (CP), accelerated phase (AP), and blast phase (BP) CML [4]
By uniting the threshold values defined by the ELN guidelines, there is a whole spectrum of BCR-ABL1 international scale (IS) “trajectories” that can be designated as Failure, Warning, or Optimal (Figure 1B)
Summary
CML is a malignant proliferation driven by a characteristic fusion gene called BCR-ABL1 [1]. The main goal of TKI therapy is to achieve a complete cytogenetic response (CCyR), defined as the lack of any detectable Ph+ cells in the bone marrow, within 12 months after starting treatment.
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