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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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REVIEW ARTICLE
Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet
Miguel A. Sanz1,
David Grimwade2,
Martin S. Tallman3,
Bob Lowenberg4,
Pierre Fenaux5,
Elihu H. Estey6,
Tomoki Naoe7,
Eva Lengfelder8,
Thomas Büchner9,
Hartmut Döhner10,
Alan K. Burnett11, and
Francesco Lo-Coco12
1 University Hospital La Fe, Valencia, Spain;
2 King's College London, London, United Kingdom;
3 Northwestern University, Chicago, IL;
4 Erasmus University Medical Center, Rotterdam, The Netherlands;
5 Hopital Avicenne, Paris, France;
6 Seattle Cancer Care Alliance, WA;
7 Nagoya University, Nagoya, Japan;
8 Universitaetsklinikum Mannheim, Heidelberg, Germany;
9 University of Munster, Munster, Germany;
10 University of Ulm, Ulm, Germany;
11 Cardiff University, Cardiff, United Kingdom; and
12 Tor Vergata University, Rome, Italy
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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