Autoimmune Lymphoproliferative Syndrome: A Case Report  

Nabil Bel Fekl , Imed Ben Ghorbel , Thouraya Ben Salem , Maria Khatib , Mohamed Habib Houman
Department of Internal Medicine, University hospital of La Rabta, Tunis, Tunisia
Author    Correspondence author
International Journal of Clinical Case Reports, 2015, Vol. 5, No. 25   doi: 10.5376/ijccr.2015.05.0025
Received: 16 May, 2015    Accepted: 18 Jun., 2015    Published: 03 Jul., 2015
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This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:

Nabil Bel Feki, Imed Ben Ghorbel, Thouraya Ben Salen and Mohamed Habib Houman, 2015, Autoimmune Lymphoproliferative Syndrome: A Case Report, International Journal of Clinical Case Reports, 5(25) 1-3 (doi: 10.5376/ijccr.2015.05.0025)

Abstract

Autoimmune lymphoproliferative syndrome is an impairment of lymphocyte apoptosis expressed by generalized non-malignant lymphoproliferation, lymphadenopathy and/or splenomegaly. We report the case of a 7-year-old male patient presentingwith relapsing generalized lymphadenopathy, splenomegaly, bicytopenia (autoimmune hemolytic anemia and thrombocytopenia), and lymphocytosis. Immunological investigations concluded to defective in vitro tumor necrosis factor receptor superfamily member 6 (Fas)-mediated apoptosis and T cells that express the alpha/beta T-cell receptor and lack of both CD4 and CD8(so-called α/β-DNT cells), and expansion of an unusual population of α/βCD3+CD4-CD8- (double-negative T cells>1%). Treatment consisted ofcorticosteroids, intravenous immunoglobulin, and cyclosporine, with favorable outcome.

Keywords
Autoimmune lymphoproliferative syndrome; lymph node; Cytopenia
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