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SHR Neuro Cancer Cardio Lipid Metab Microb

Rensing-Ehl, A; Warnatz, K; Fuchs, S; Schlesier, M; Salzer, U; Draeger, R; Bondzio, I; Joos, Y; Janda, A; Gomes, M; Abinun, M; Hambleton, S; Cant, A; Shackley, F; Flood, T; Waruiru, C; Beutel, K; Siepermann, K; Dueckers, G; Niehues, T; Wiesel, T; Schuster, V; Seidel, MG; Minkov, M; Sirkiä, K; Kopp, MV; Korhonen, M; Schwarz, K; Ehl, S; Speckmann, C.
Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency.
Clin Immunol. 2010; 137(3):357-365 Doi: 10.1016/j.clim.2010.08.008
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Co-authors Med Uni Graz: Seidel Markus
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Abstract:: Autoimmune lymphoproliferative syndrome (ALPS) is mainly caused by defects in the CD95 pathway. Raised CD3+TCRαβ+CD4-CD8- double negative T cells and impaired T cell apoptosis are hallmarks of the disease. In contrast, the B cell compartment has been less well studied. We found an altered distribution of B cell subsets with raised transitional B cells and reduced marginal zone B cells, switched memory B cells and plasma blasts in most of 22 analyzed ALPS patients. Moreover, 5 out of 66 ALPS patients presented with low IgG and susceptibility to infection revealing a significant overlap between ALPS and common variable immunodeficiency (CVID). In patients presenting with lymphoproliferation, cytopenia, hypogammaglobulinemia and impaired B cell differentiation, serum biomarkers were helpful in addition to apoptosis tests for the identification of ALPS patients. Our observations may indicate a role for apoptosis defects in some diseases currently classified as CVID.
Find related publications in this database (using NLM MeSH Indexing): Adolescent -; Adult -; Agammaglobulinemia - immunology; Antigens, CD95 - blood; Apoptosis -; Autoimmune Lymphoproliferative Syndrome - diagnosis; B-Lymphocytes - immunology; Biological Markers - blood; Child -; Child, Preschool -; Common Variable Immunodeficiency - diagnosis; Diagnosis, Differential -; Fas Ligand Protein - blood; Flow Cytometry -; Humans -; Immunoglobulin G - blood; Interleukin-10 - blood; Middle Aged -; Monocytes - immunology; Phenotype -; T-Lymphocytes - immunology; Vitamin B 12 - blood
Find related publications in this database (Keywords): ALPS; CVID; B cells; Immunoglobulins