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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Keywords ECG abnormality, QT prolongation, allogeneic stem cell transplantation, arsenic trioxide, autologous stem cell transplantation, brain radiation, cancer combination chemotherapy, central nervous system metastasis, cytarabine, daunorubicin, diagnostic approach route, diagnostic test, drug safety, electrolyte disturbance, evidence based practice, first trimester pregnancy, fluorescence in situ hybridization, gemtuzumab ozogamicin, genetic risk, genetic variability, hematopoietic stem cell transplantation, human, hydrocortisone, hyperglycemia, hypokalemia, idarubicin, immunohistochemistry, karyotyping, leukemia relapse, leukemia remission, medical specialist, methotrexate, mitoxantrone, patient monitoring, patient selection, practice guideline, pregnant woman, priority journal, promyelocytic leukemia, retinoic acid, reverse transcription polymerase chain reaction, review, risk benefit analysis, second trimester pregnancy, syncope, teratogenicity, therapy effect, torsade des pointes, treatment indication, treatment outcome, treatment response
Persistent URL dx.doi.org/10.1182/blood-2008-04-150250, hdl.handle.net/1765/18239
Citation
Sanz, M.A, Grimwade, D, Tallman, M.S, Löwenberg, B, Fenaux, P, Estey, E.H, … Lo-Coco, F. (2009). Management of acute promyelocytic leukemia: Recommendations from an expert panel on behalf of the European LeukemiaNet. Blood, 113(9), 1875–1891. doi:10.1182/blood-2008-04-150250