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Characterisation of the PML/RARα Rearrangement Associated with t(15;17) Acute Promyelocytic Leukaemia

  • Chapter
Chromosomal Translocations and Oncogenic Transcription Factors

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 220))

Abstract

Acute promyelocytic leukaemia (APL; FAB AML M3, Bennett et al. 1976) represents a unique example of a disease in which a successful treatment approach, in the form of all-trans retinoic acid (ATRA), has been developed that directly addresses and overcomes the causative molecular abnormality. For over a decade, retinoids have been noted to possess therapeutic activity which is virtually specific to the acute promyelocytic form of acute myeloid leukaemia (AML) (Breitman et al. 1981). Subsequent clinical trials have shown that ATRA can achieve remission rates of over 90% in APL (Huang et al. 1988; Castaigne et al. 1990; CHOMIENNE et al. 1990), representing an apparent significant improvement on results obtained with conventional chemotherapy; indeed a number of patients in these studies were chemoresistant or treated in relapse. Parallel in vitro studies have demonstrated that remission is achieved by terminal differentiation of the leukaemic clone rather than by a cytotoxic effect (Huang et al. 1988; Castaigne et al. 1990; Chomienne et al. 1990). This has been confirmed using clonal analysis of APL blasts and peripheral blood neutrophils following ATRA therapy (ELLIOTT et al. 1992).

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Grimwade, D., Solomon, E. (1997). Characterisation of the PML/RARα Rearrangement Associated with t(15;17) Acute Promyelocytic Leukaemia. In: Rauscher, F.J., Vogt, P.K. (eds) Chromosomal Translocations and Oncogenic Transcription Factors. Current Topics in Microbiology and Immunology, vol 220. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-60479-9_6

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