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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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)

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.

Autoimmune lymphoproliferative syndrome; lymph node; Cytopenia