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Blood, 26 February 2009, Vol. 113, No. 9, pp. 1875-1891.
Prepublished online as a Blood First Edition Paper on September 23, 2008; DOI 10.1182/blood-2008-04-150250.


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Submitted April 9, 2008
Accepted August 26, 2008

Guidelines on the management of acute promyelocytic leukemia: Recommendations from an expert panel on behalf of the European LeukemiaNet

Miguel A. Sanz*, David Grimwade, Martin S Tallman, Bob Lowenberg, Pierre Fenaux, Elihu H. Estey, Tomoki Naoe, Eva Lengfelder, Thomas Buchner, Hartmut Dohner, Alan K. Burnett, and Francesco Lo-Coco

University Hospital La Fe, Valencia, Spain
King's College London, London, United Kingdom
Northwestern University, Chicago, IL, United States
Erasmus University Medical Center, Rotterdam, Netherlands
Hopital Avicenne, Paris, France
Seattle Cancer Care Alliance, Seattle, WA, United States
Nagoya University, Nagoya, Japan
Universitaetsklinikum Mannheim, Mannheim, Germany
University of Munster, Munster, Germany
University of Ulm, Ulm, Germany
Cardiff University, Cardiff, United Kingdom
Tor Vergata University, Rome, Italy

* Corresponding author; email: msanz{at}uv.es.

The introduction of all-trans retinoic acid (ATRA) and, more recently, arsenic trioxide (ATO) into the therapy of acute promyelocytic leukemia (APL) has revolutionized the management and outcome of this disease. Several treatment strategies using these agents, usually in combination with chemotherapy, but also without or with minimal use of cytotoxic agents, have provided excellent therapeutic results. Cure of APL patients, however, is also dependent on peculiar aspects related to the management and supportive measures that are crucial to counteract life-threatening complications associated with the disease biology and molecularly targeted treatment. The European LeukemiaNet recently appointed an international panel of experts to develop evidence- and expert opinion-based guidelines on the diagnosis and management of APL. Together with providing current indications on genetic diagnosis, modern risk-adapted front-line therapy and salvage treatment, the review contains specific recommendations for the identification and management of most important complications such as the bleeding disorder, APL differentiation syndrome, QT prolongation and other ATRA and ATO related toxicities, as well as for molecular assessment of response to treatment. Finally, the approach to special situations is also discussed including management of APL in children, elderly patients and pregnant women.


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