Chronic Myeloid Leukemia: A Handbook for Hematologists and by Tariq I. Mughal

By Tariq I. Mughal

Highly recommended, BMA scientific e-book Awards 2014

Chronic myeloid leukemia is a clonal ailment that effects from an received genetic switch in one pluripotential hemopoietic stem cellphone. Efforts to confront this disorder were assisted by means of advances in mobile and molecular biology and the rate at which those findings have reworked the healing set of rules. however, there's a few uncertainty near to how most sensible to evaluate efficacy of remedy, specially near to the main acceptable surrogate markers for total survival. From the point of view of biology and medical administration, this guide presents an authoritative cutting-edge assessment of present and constructing progress.

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Example text

About half of the patients in the CP transformed directly into BC and the remainder did so following a period of accelerated phase. This natural history of CML appears to have changed significantly in patients treated with TKIs, with the majority not progressing beyond the CP, especially if they remain in CCyR beyond two years of imatinib treatment. There have been a few reports of patients who achieved a CCyR and subsequently relapsed directly into advanced phase, in particular BC. The risk appears to be the highest for patients presenting in late CP at the time when imatinib was started.

19 3 Cytogenetics, molecular anatomy, and molecular biology of chronic myeloid leukemia INTRODUCTION The Philadelphia (Ph) chromosome is an acquired cytogenetic abnormality present in all leukemic cells of the myeloid lineage and in some B cells and T cells in patients with CML. It is formed as a result of a reciprocal translocation of genetic material of chromosomes 9 and 22 (Figs. 1). This balanced translocation results in a BCR–ABL1 fusion gene on the Ph chromosome and also a “reciprocal” fusion gene, designated ABL–BCR, on the derivative 9q chromosome (der 9q+).

Cytogenetics, molecular anatomy, and molecular biology of chronic myeloid leukemia are reviewed. CYTOGENETICS AND MOLECULAR ANATOMY The Ph chromosome is an acquired cytogenetic abnormality that characterizes all leukemic cells in CML. It is formed as a result of a reciprocal translocation of chromosomal material between the long arms of chromosome 22 and chromosome 9, t(9; 22) (q34; q11). This balanced translocation results in a BCR–ABL1 fusion gene on the Ph chromosome (see below) and also a “reciprocal” fusion gene, designated ABL–BCR, on the derivative 9q chromosome.

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