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Blood, 22 January 2009, Vol. 113, No. 4, pp. 775-783. Prepublished online as a Blood First Edition Paper on October 22, 2008; DOI 10.1182/blood-2008-07-168617. This Article Full Text (PDF) Supplemental Appendix All Versions of this Article: blood-2008-07-168617v1 113/4/775    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Montesinos, P. Articles by Sanz, M. A Search for Related Content PubMed PubMed Citation Articles by Montesinos, P. Articles by Sanz, M. A Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted July 16, 2008 Accepted September 18, 2008 Differentiation syndrome in patients with acute promyelocytic leukemia treated with all-transretinoic acid and anthracycline chemotherapy: characteristics, outcome and prognostic factors Pau Montesinos, Juan M. Bergua, Edo Vellenga, Chelo Rayon, Ricardo Parody, Javier de la Serna, Angel Leon, Jordi Esteve, Gustavo Milone, Guillermo Deben, Concha Rivas, Marcos Gonzalez, Mar Tormo, Joaquin Diaz-Mediavilla, Jose D Gonzalez, Silvia Negri, Elena Amutio, Salut Brunet, Bob Lowenberg, and Miguel A Sanz* Department of Medicine of the Universitat Autonoma de Barcelona, Hospital Universitario La Fe, Valencia, Spain Hospital San Pedro de Alcantar, Caceres, Spain University Hospital, Groningen, Netherlands Hospital Central de Asturias, Oviedo, Spain Hospital Universitario Virgen del Rocio, Sevilla, Spain Hospital 12 de Octubre, Madrid, Spain Hospital General, Jerez de la Frontera, Spain Hospital Clinic, Barcelona, Spain Fundaleu, Buenos Aires, Argentina Hospital Juan Canalejo, La Coruna, Spain Hospital General, Alicante, Spain Hospital Universitario, Salamanca, Spain Hospital Clinico Universitario, Valencia, Spain Hospital Clinico San Carlos, Madrid, Spain Hospital Insular, Las Palmas, Spain Hospital Carlos Haya, Malaga, Spain Hospital Cruces, Baracaldo, Spain Hospital Sant Pau, Barcelona, Spain Erasmus University Medical Center, Rotterdam, Netherlands Hospital Universitario La Fe, Valencia, Spain * Corresponding author; email: msanz{at}uv.es. Differentiation syndrome (DS) can be a life-threatening complication in patients with acute promyelocytic leukemia (APL) undergoing induction therapy with all-trans retinoic acid (ATRA). Detailed knowledge about the differentiation syndrome has remained limited. We present an analysis of the incidence, characteristics, prognostic factors, and outcome of DS in 739 APL patients treated with ATRA plus idarubicin in two consecutive trials (PETHEMA LPA96 and LPA99). Overall, 183 patients (24.8%) experienced DS, 93 with a severe form (12.6%) and 90 with a moderate form (12.2%). Severe but not moderate DS was associated with an increase in mortality. A bimodal incidence of DS was observed, with peaks occurring in the first and third weeks after the start of ATRA therapy. Multivariate analysis identified presenting WBC count of > 5 109/L and abnormal serum creatinine level correlating with an increased risk of development of severe DS. Patients receiving systematic prednisone prophylaxis (LPA99 trial) in contrast to those receiving selective prophylaxis with dexamethasone (LPA96 trial) had a lower incidence of severe DS. Patients developing severe DS showed a reduced seven-year relapse-free survival in the LPA96 trial (60% vs 85%, P = 0.003), but this difference was not apparent in the LPA99 trial (86% vs 88%). CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: K. Furugaki, K. Pokorna, C. Le Pogam, M. Aoki, M. Reboul, V. Bajzik, P. Krief, A. Janin, M.-E. Noguera, R. West, et al. DNA vaccination with all-trans retinoic acid treatment induces long-term survival and elicits specific immune responses requiring CD4+ and CD8+ T-cell activation in an acute promyelocytic leukemia mouse model Blood, January 21, 2010; 115(3): 653 - 656. [Abstract] [Full Text] [PDF] M. Luesink, J. L. A. Pennings, W. M. Wissink, P. C. M. Linssen, P. Muus, R. 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