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Acute promyelocytic leukemia: PML/RARalpha and the leukemic stem cell.

Puccetti, E and Ruthardt, M ORCID: https://orcid.org/0000-0003-1021-3811 2004. Acute promyelocytic leukemia: PML/RARalpha and the leukemic stem cell. Leukemia 18 , 1169–1175. 10.1038/sj.leu.2403367

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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/RARalpha fusion protein. PML/RARalpha 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/RARalpha on the biology of the hematopoietic stem cell. Moreover, the importance of the blockage of t-RA signaling by the PML/RARalpha for the pathogenesis of APL is discussed, taking the relevance of the t-RA signaling pathway for the global hematopoiesis into account.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Publisher: Springer Nature [academic journals on nature.com]
ISSN: 0887-6924
Date of Acceptance: 23 February 2004
Last Modified: 27 Feb 2024 14:00
URI: https://orca.cardiff.ac.uk/id/eprint/166059

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