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Blood, 1 March 2008, Vol. 111, No. 5, pp. 2556-2562. Prepublished online as a Blood First Edition Paper on December 18, 2007; DOI 10.1182/blood-2007-08-106211. This Article Full Text Full Text (PDF) Supplemental Appendix All Versions of this Article: blood-2007-08-106211v1 111/5/2556    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map 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 Gadner, H. Articles by Ladisch, S. Search for Related Content PubMed PubMed Citation Articles by Gadner, H. Articles by Ladisch, S. Related Collections Clinical Trials and Observations Related Articles in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification Helmut Gadner1, Nicole Grois1, Ulrike Pötschger1, Milen Minkov1, Maurizio Aricò2, Jorge Braier3, Valerie Broadbent4, Jean Donadieu5, Jan-Inge Henter6, Robert McCarter7, Stephan Ladisch7, for the Histiocyte Society 1 Children's Cancer Research Institute, St Anna Children's Hospital, Vienna, Austria; 2 Pediatric Hematology/Oncology, University of Palermo, Palermo, Italy; 3 Hospital Nacional de Pediatria J. Garrahan, Buenos Aires, Argentina; 4 Addenbrookes Hospital, Cambridge, United Kingdom; 5 Assistance-Publique Hôpitaux de Paris, Hopital Trousseau, Paris, France; 6 Childhood Cancer Research Unit, Department of Women and Child, Karolinska University Hospital, Stockholm, Sweden; and 7 Children's Research Institute, Children's National Medical Center, Washington, DC Multisystem Langerhans cell histiocytosis (MS-LCH) is associated with high mortality when patients have risk organ involvement (RO+) or are younger than 2 years. In an international randomized trial, LCH-II, we intensified their treatment: arm A consisted of 6 weeks of daily prednisone and weekly vinblastine followed by 18 weeks of daily 6-mercaptopurine with vinblastine/prednisone pulses; etoposide was added in arm B. Considering all 193 randomized risk patients, there were similar outcomes: rapid (6 weeks) response (arm A vs arm B: 63%/71%), 5-year survival probability (74%/79%), disease reactivation frequency (46%/46%), and permanent consequences (43%/37%). However, (1) patients younger than 2 years without RO involvement (RO–) had 100% survival and uniformly high (> 80%) rapid response, (2) RO+ patients not responding within 6 weeks had highest mortality, and (3) importantly, the more intensive arm B reduced mortality in RO+ patients (relative hazard rate, accounting for differences in risk organ involvement, of 0.54; 95% CI = 0.29-1.00). Finally, comparison of RO+ patients in LCH-I and LCH-II confirmed that increasing treatment intensity increased rapid responses (from 43% in arm A LCH-I to 68% in arm B LCH-II; P = .027) and reduced mortality (from 44% in arm A LCH-I to 27% in arm B LCH-II; P = .042). We conclude that intensified treatment significantly increases rapid response and reduces mortality in risk MS-LCH. This trial was registered at http://www.controlled-trials.com as no. ISRCTN57679341 [controlled-trials.com] . CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Articles in Blood Online: About reporting clinical trials Jean-Louis Bernard, Julie Berbis, Laurent Boyer, and Vincent Pradel Blood 2008 112: 3527. [Full Text] [PDF] Response: Design and reporting of clinical trials in LCH Stephan Ladisch, Robert McCarter, Ulrike Pötschger, and Helmut Gadner Blood 2008 112: 3528. [Full Text] [PDF] This article has been cited by other articles: K Windebank and V Nanduri Langerhans cell histiocytosis Arch. Dis. Child., November 1, 2009; 94(11): 904 - 908. [Abstract] [Full Text] [PDF] E. Derenzini, M. P. Fina, V. Stefoni, C. Pellegrini, F. Venturini, A. Broccoli, L. Gandolfi, S. Pileri, S. Fanti, E. Lopci, et al. MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients Ann. Onc., October 27, 2009; (2009) mdp455v1. [Abstract] [Full Text] [PDF] B. A. Degar and B. J. Rollins Langerhans cell histiocytosis: malignancy or inflammatory disorder doing a great job of imitating one? Dis. Model. Mech., September 1, 2009; 2(9-10): 436 - 439. [Abstract] [Full Text] [PDF] J.-L. Bernard, J. Berbis, L. Boyer, and V. Pradel About reporting clinical trials Blood, October 15, 2008; 112(8): 3527 - 3527. [Full Text] [PDF] S. Ladisch, R. McCarter, U. Potschger, and H. Gadner Response: Design and reporting of clinical trials in LCH Blood, October 15, 2008; 112(8): 3528 - 3528. [Full Text] [PDF]

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