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Blood, 1 February 2005, Vol. 105, No. 3, pp. 994-996. Prepublished online as a Blood First Edition Paper on October 19, 2004; DOI 10.1182/blood-2004-07-2965. This Article Full Text (PDF) All Versions of this Article: 2004-07-2965v1 105/3/994    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 Milone, M. Articles by Nichols, K. E Search for Related Content PubMed PubMed Citation Articles by Milone, M. Articles by Nichols, K. E Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted August 3, 2004 Accepted October 4, 2004 Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell directed therapy Michael Milone, Donald E Tsai, Richard L Hodinka, Lewis B Silverman, Alejandro Malbran, Mariusz A Wasik, and Kim E Nichols* Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, USA Hematologic Malignancies Program, University of Pennsylvania, Philadelphia, PA, USA Clinical Virology Laboratory, Children's Hospital of Philadelphia, Philadelphia, PA, USA; Pediatrics and Pathology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA, USA; Pediatric Hematology-Oncology, Children's Hospital, Boston, MA, USA Allergy and Immunology, Hospital Britanico, Buenos Aires, Argentina Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, PA, USA * Corresponding author; email: nicholsk{at}email.chop.edu. X-linked lymphoproliferative disease (XLP) is a congenital immunodeficiency that is characterized by an abnormal immune response to primary Epstein-Barr virus (EBV) infection. After EBV exposure, affected patients often develop fulminant infectious mononucleosis (FIM), a life-threatening condition marked by the uncontrolled expansion and activation of T and B lymphocytes and macrophages. We hypothesized that the rapid elimination of B cells immediately following EBV exposure might reduce the severity of primary EBV infection in XLP patients. To test this possibility, we administered the anti-CD20 antibody rituximab to 2 patients who presented with acute infection. Following treatment, both patients exhibited a complete resolution of symptoms and no longer demonstrated detectable EBV DNA within circulating lymphocytes. Moreover, neither patient has developed FIM or lymphoma in over 2 years of follow-up. These data suggest that the pre-emptive use of B cell-directed therapy may reduce the morbidity and mortality of primary EBV infection in XLP-affected individuals. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: J. Pachlopnik Schmid, D. Moshous, N. Boddaert, B. Neven, L. Dal Cortivo, M. Tardieu, M. Cavazzana-Calvo, S. Blanche, G. de Saint Basile, and A. Fischer Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients Blood, July 2, 2009; 114(1): 211 - 218. [Abstract] [Full Text] [PDF] N. Mahlaoui, M. Ouachee-Chardin, G. de Saint Basile, B. Neven, C. Picard, S. Blanche, and A. Fischer Immunotherapy of Familial Hemophagocytic Lymphohistiocytosis With Antithymocyte Globulins: A Single-Center Retrospective Report of 38 Patients Pediatrics, September 1, 2007; 120(3): e622 - e628. [Abstract] [Full Text] [PDF] M. Mischler, G. M. Fleming, T. P. Shanley, L. Madden, J. Levine, V. Castle, A. H. Filipovich, and T. T. Cornell Epstein-Barr Virus-Induced Hemophagocytic Lymphohistiocytosis and X-Linked Lymphoproliferative Disease: A Mimicker of Sepsis in the Pediatric Intensive Care Unit Pediatrics, May 1, 2007; 119(5): e1212 - e1218. [Abstract] [Full Text] [PDF] M. V. Lemas and R. F. Ambinder The kiss that scars Blood, July 1, 2006; 108(1): 8 - 8. [Full Text] [PDF] O. Thaunat, M.-F. Mamzer-Bruneel, F. Agbalika, F. Valensi, M. Venditto, C. Lebbe, C. Frances, R. Kania, L. Chatenoud, C. Antoine, et al. Severe human herpesvirus-8 primary infection in a renal transplant patient successfully treated with anti-CD20 monoclonal antibody. Blood, April 1, 2006; 107(7): 3009 - 3010. [Full Text] [PDF] H. Williams and D. H. Crawford Epstein-Barr virus: the impact of scientific advances on clinical practice Blood, February 1, 2006; 107(3): 862 - 869. [Abstract] [Full Text] [PDF]

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