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Prepublished online as a Blood First Edition Paper on June 7, 2002; DOI 10.1182/blood-2002-01-0172. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-01-0172v1 100/7/2367    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 Henter, J.-I. Articles by Janka, G. Search for Related Content PubMed PubMed Citation Articles by Henter, J.-I. Articles by Janka, G. Related Collections Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 1 October 2002, Vol. 100, No. 7, pp. 2367-2373 CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation Jan-Inge Henter, AnnaCarin Samuelsson-Horne, Maurizio Aricò, R. Maarten Egeler, Göran Elinder, Alexandra H. Filipovich, Helmut Gadner, Shinsaku Imashuku, Diane Komp, Stephan Ladisch, David Webb, and Gritta Janka for the Histiocyte Society From the Childhood Cancer Research Unit, Karolinska Institutet, Department of Pediatric Hematology and Oncology, Karolinska Hospital, Stockholm, Sweden; the Department of Pediatrics, Stockholm Söder Hospital, Karolinska Institutet, Stockholm, Sweden; the Onco Ematologia Pediatrica, Ospedale dei Bambini G di Cristina, Palermo, Italy; the Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands; the Children's Hospital Medical Center, Cincinnati, OH; the St Anna Children's Hospital, Vienna, Austria; the Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan; the Department of Pediatrics, Yale University School of Medicine, New Haven, CT; the Children's Research Institute, Washington, DC; the Great Ormond Street Hospital, London, United Kingdom; the Department of Hematology and Oncology, Children's University Hospital, Hamburg, Germany. Hemophagocytic lymphohistiocytosis (HLH) comprises familial (primary) hemophagocytic lymphohistiocytosis (FHL) and secondary HLH (SHLH), both clinically characterized by fever, hepatosplenomegaly, and cytopenia. FHL, an autosomal recessive disease invariably fatal when untreated, is associated with defective triggering of apoptosis and reduced cytotoxic activity, resulting in a widespread accumulation of T lymphocytes and activated macrophages. In 1994 the Histiocyte Society initiated a prospective international collaborative therapeutic study (HLH-94), aiming at improved survival. It combined chemotherapy and immunotherapy (etoposide, corticosteroids, cyclosporin A, and, in selected patients, intrathecal methotrexate), followed by bone marrow transplantation (BMT) in persistent, recurring, and/or familial disease. Between July 1, 1994, and June 30, 1998, 113 eligible patients aged no more than 15 years from 21 countries started HLH-94. All had either an affected sibling (n = 25) and/or fulfilled the Histiocyte Society diagnostic criteria. At a median follow-up of 3.1 years, the estimated 3-year probability of survival overall was 55% (95% confidence interval ± 9%), and in the familial cases, 51% (± 20%). Twenty enrolled children were alive and off therapy for more than 12 months without BMT. For patients who received transplants (n = 65), died prior to BMT (n = 25), or were still on therapy (n = 3), the 3-year survival was 45% (± 10%). The 3-year probability of survival after BMT was 62% (± 12%). HLH-94 is very effective, allowing BMT in most patients. Survival of children with HLH has been greatly improved. © 2002 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. Santoro, S. Cannella, A. Trizzino, G. Bruno, C. De Fusco, L. D. Notarangelo, D. Pende, G. M. Griffiths, and M. 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	Copyright © 2002 by American Society of Hematology         Online ISSN: 1528-0020