Idiopathic CD4+ T cell lymphocytopenia with the absence of B cells and CD8+28+ cells in peripheral blood.
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 p56 (lck) 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.
Kutukculer
N
N
Department of Pediatrics, Faculty of Medicine, Ege University, 35100 Bornova, Izmir, Turkey. Necil@med.ege.edu.tr
Aksu
G
G
Genel
F
F
Ozturk
C
C
D001402:B-Lymphocytes / Q000276:immunology; D018106:CD28 Antigens / Q000378:metabolism; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D019860:Lymphocyte Specific Protein Tyrosine Kinase p56(lck) / Q000378:metabolism; D008231:Lymphopenia / Q000276:immunology* / Q000378:metabolism / Q000628:therapy; D016328:NF-kappa B / Q000378:metabolism; D016176:T-Lymphocyte Subsets / Q000276:immunology
D018106:CD28 Antigens; D016756:Immunoglobulins, Intravenous; D016328:NF-kappa B; D019860:Lymphocyte Specific Protein Tyrosine Kinase p56(lck)