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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 (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 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 Related Articles in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted August 10, 2007 Accepted December 4, 2007 Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification Helmut Gadner, Nicole Grois, Ulrike Potschger, Milen Minkov, Maurizio Arico, Jorge Braier, Valerie Broadbent, Jean Donadieu, Jan-Inge Henter, Robert McCarter, and Stephan Ladisch* Children's Cancer Research Institute, St Anna Children's Hospital, Vienna, Austria Pediatric Hematology/Oncology, University of Palermo, Palermo, Italy Hospital Nacional de Pediatria J Garrahan, Buenos Aires, Argentina Addenbrookes Hospital, Cambridge, United Kingdom Assistance-Publique Hopitaux de Paris, Hopital Trousseau, Paris, France Karolinska University Hospital, Stockholm, Sweden Children's Research Institute, Children's National Medical Center, Washington, DC, United States * Corresponding author; email: sladisch{at}cnmc.org. Multisystem Langerhans cell histiocytosis (MS-LCH) is associated with high mortality when patients have risk organ involvement (RO+) or age <2 years. In an international randomized trial, LCH-II, we intensified their treatment: Arm A consisted of 6-weeks daily prednisone and weekly vinblastine followed by 18-weeks 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 (A vs. B, 63%/71%), 5-year survival probability (74%/79%), disease reactivation frequency (46%/46%), and permanent consequences (43%/37%). However, (i) patients <2 years old without RO involvement (RO-) had 100% survival and uniformly high (>80%) rapid response, (ii) RO+ patients not responding within 6 weeks had highest mortality, and (iii) 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%, Arm A LCH-I, to 68%, Arm B LCH-II; p=0.027) and reduced mortality (from 44%, Arm A LCH-I, to 27%, Arm B, LCH-II; p=0.042). We conclude that intensified treatment significantly increases rapid response and reduces mortality in risk MS-LCH. This trial was registered at www.controlled-trials.com as #ISRCTN57679341. 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: H. Gadner Treatment of adult-onset Langerhans cell histiocytosis--is it different from the pediatric approach? Ann. Onc., November 25, 2009; (2009) mdp540v1. [Full Text] [PDF] 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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