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Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma : An Adjunctive Diagnostic Role for Monitoring EBV Viremia?
Joint Authors
Source
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-07-25
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Pace, Romina& Vinh, Donald C.. 2013. Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma : An Adjunctive Diagnostic Role for Monitoring EBV Viremia?. Case Reports in Immunology،Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-456952
Modern Language Association (MLA)
Pace, Romina& Vinh, Donald C.. Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma : An Adjunctive Diagnostic Role for Monitoring EBV Viremia?. Case Reports in Immunology No. 2013 (2013), pp.1-5.
https://search.emarefa.net/detail/BIM-456952
American Medical Association (AMA)
Pace, Romina& Vinh, Donald C.. Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma : An Adjunctive Diagnostic Role for Monitoring EBV Viremia?. Case Reports in Immunology. 2013. Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-456952
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-456952