Idiopathic CD4+ T cell lymphocytopenia with the absence of B cells and CD8+28+ cells in peripheral blood |
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Authors: | Kutukculer N Aksu G Genel F Ozturk C |
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Affiliation: | (1) Department of Pediatrics, Faculty of Medicine, Ege University, 35100 Bornova, İzmir, Turkey. Necil@med.ege.edu.tr, TR |
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Abstract: | The absence of B cells and a severe decrease in CD8+28+ cells were observed in two female children with CD4+ T cell lymphocytopenia. Idiopathic (primary) CD4+ lymphocytopenia is a rare entity and its pathogenesis and genetics are not yet known. The literature was reviewed, in particular for severe alterations in B and CD8+28+ cells and for the role of NF-kappa B and p56lck in the immunopathogenesis. Whether the underlying mechanism in idiopathic CD4+ lymphocytopenia is found or not, these patients who present with severe symptoms of a combined immunodeficiency must be treated with intravenous immunoglobin regularly until they have a compatible donor for bone marrow transplantation. Received: 4 May 2001 / Accepted: 10 October 2002 Correspondence to N. Kutukculer |
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Keywords: | Primary (idiopathic) CD4+ lymphocytopenia Intravenous immunoglobuline Bone marrow transplantation |
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