Differentiation syndrome in patients with acute promyelocytic leukemia treated with all- trans retinoic acid and anthracycline chemotherapy: Characteristics, outcome, and prognostic factors

Pau Montesinos, Juan M. Bergua, Edo Vellenga, Chelo Rayón, Ricardo Parody, Javier De La Serna, Angel León, Jordi Esteve, Gustavo Milone, Guillermo Debén, Concha Rivas, Marcos González, Mar Tormo, Díaz Mediavilla Joaquín, Jose D. González, Silvia Negri, Elena Amutio, Salut Brunet, Bob Lowenberg, Miguel A. Sanz

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209 Citations (Scopus)

Abstract

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 knowl- edge about DS has remained limited. We present an analysis of the incidence, char- acteristics, prognostic factors, and out- come of 739 APL patients treated with ATRA plus idarubicin in 2 consecutive trials (Programa Espanol de Tratamientos en Hematologíc [PETHEMA] LPA96 and LPA99). Overall, 183 patients (24.8%) ex- perienced 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. A multivariate analysis indicated that a WBC count greater than 5 x 109/L and an abnor- mal serum creatinine level correlated with an increased risk of developing severe DS. Patients receiving systematic pred- nisone prophylaxis (LPA99 trial) in con- trast to those receiving selective prophy- laxis with dexamethasone (LPA96 trial) had a lower incidence of severe DS. Pa- tients developing severe DS showed a reduced 7-year relapse-free survival in the LPA96 trial (60% vs 85%, P = .003), but this difference was not apparent in the LPA99 trial. © 2009 by The American Society of Hematology.
Original languageEnglish
Pages (from-to)775-783
JournalBlood
Volume113
Issue number4
DOIs
Publication statusPublished - 22 Jan 2009

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