Case Reports in Immunology
Volume 2013 (2013), Article ID 245893, 5 pages
http://dx.doi.org/10.1155/2013/245893
Case Report
Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma: An Adjunctive Diagnostic Role for Monitoring EBV Viremia?
1Department of Medicine, McGill University Health Centre, Montreal, QC, H3G 1A4, Canada
2Division of Infectious Diseases, Division of Allergy & Clinical Immunology (Department of Medicine), Department of Medical Microbiology, Department of Human Genetics, McGill University Health Centre, Montreal General Hospital, 1650 Cedar Avenue, Rm A5-156, Montreal, QC, H3G 1A4, Canada
2Division of Infectious Diseases, Division of Allergy & Clinical Immunology (Department of Medicine), Department of Medical Microbiology, Department of Human Genetics, McGill University Health Centre, Montreal General Hospital, 1650 Cedar Avenue, Rm A5-156, Montreal, QC, H3G 1A4, Canada
Received 30 May 2013; Accepted 2 July 2013
Academic Editors: N. Martinez-Quiles, A. E. Tebo, M. Trendelenburg, and A. Vojdani
Copyright © 2013 Romina Pace and Donald C. Vinh. This is an open access article distributed under theCreative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Background. Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of lymphocyte homeostasis due to defects in FAS-mediated apoptosis. ALPS is characterized by childhood onset of chronic lymphadenopathy and splenomegaly, autoimmunity, an expanded population of double-negative T cells (DNTCs), and an increased risk of lymphoma. This propensity for lymphoma in ALPS is not well understood. It is possible that lymphomagenesis in some of these patients may result from Epstein-Barr virus (EBV) infection exploiting the defective T-cell surveillance resulting from impaired FAS-mediated apoptosis. Case Presentation. We report the first case, to our knowledge, of lymphoma in a patient with ALPS that was clinically heralded by progressively increasing EBV viremia. We discuss its practical implications and the possible immune pathways involved in the increased risk for EBV-associated lymphoproliferative disorders in ALPS patients. Conclusion. In patients with ALPS, distinguishing chronic lymphadenopathy from emerging lymphoma is difficult, with few practical recommendations available. This case illustrates that, at least for some patients, monitoring for progressively increasing EBV viremia may be useful.
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