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Acute promyelocytic leukemia: PML/RARα and the leukemic stem cell

Leukemia volume 18, pages 1169–1175 (2004)Cite this article

Abstract

Acute promyelocytic leukemia (APL) is distinguished from other acute myeloid leukemias (AMLs) by cytogenetic, clinical, as well as biological characteristics. The hallmark of APL is the t(15;17), which leads to the expression of the PML/RARα fusion protein. PML/RARα is the central leukemia-inducing lesion in APL and is directly targeted by all trans retinoic acid (t-RA) as well as by arsenic, both compounds able to induce complete remissions. This review focuses on potential stem cell involvement in APL outlining the knowledge about the APL-initiating stem cell and the influence of PML/RARα on the biology of the hematopoietic stem cell. Moreover, the importance of the blockage of t-RA signaling by the PML/RARα for the pathogenesis of APL is discussed, taking the relevance of the t-RA signaling pathway for the global hematopoiesis into account.

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Acknowledgements

We are grateful to Dr Reinhard Henschler and Dr Simone Boehrer for helpful discussions and critical reading of the manuscript. Martin Ruthardt is supported by grants from Deutsche Forschungsgemeinschaft (DFG RU 728/2-2), Dr Mildred-Scheel-Stiftung der Deutschen Krebshilfe e.V. (10-1916 Ru2), and Wilhelm Sander-Stiftung (2001-026.1).

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  1. Med. Klinik III/Abtl. Hämatologie, Labor für Experimentelle Hämatologie, Johann Wolfgang Goethe-Universität, Frankfurt, Germany

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Puccetti, E., Ruthardt, M. Acute promyelocytic leukemia: PML/RARα and the leukemic stem cell. Leukemia 18, 1169–1175 (2004). https://doi.org/10.1038/sj.leu.2403367

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