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231223s2010 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2010.08.008
|2 doi
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|a pubmed24n0672.xml
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|a eng
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|a Rensing-Ehl, A
|e verfasserin
|4 aut
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|a Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency
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|c 2010
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
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|2 rdamedia
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|a ƒa Online-Ressource
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|a Date Completed 26.11.2010
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|a Date Revised 20.10.2021
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2010 Elsevier Inc. All rights reserved.
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|a 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
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|a Comparative Study
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|a Journal Article
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|a Research Support, Non-U.S. Gov't
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|a Biomarkers
|2 NLM
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|a Fas Ligand Protein
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|a Immunoglobulin G
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|a fas Receptor
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|a Interleukin-10
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|a 130068-27-8
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|a Warnatz, K
|e verfasserin
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|a Fuchs, S
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|a Schlesier, M
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|a Salzer, U
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|a Draeger, R
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|a Bondzio, I
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|a Joos, Y
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|a Janda, A
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|a Gomes, M
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|a Abinun, M
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|a Hambleton, S
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|a Cant, A
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|a Shackley, F
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|a Flood, T
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|a Waruiru, C
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|a Beutel, K
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|a Siepermann, K
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|a Dueckers, G
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|a Niehues, T
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|a Wiesel, T
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|a Schuster, V
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|a Seidel, M G
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|a Minkov, M
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|a Sirkiä, K
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|a Kopp, M V
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|a Korhonen, M
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|a Schwarz, K
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|a Ehl, S
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|a Speckmann, C
|e verfasserin
|4 aut
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 137(2010), 3 vom: 24. Dez., Seite 357-65
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|x 1521-7035
|7 nnns
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|g volume:137
|g year:2010
|g number:3
|g day:24
|g month:12
|g pages:357-65
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|u http://dx.doi.org/10.1016/j.clim.2010.08.008
|3 Volltext
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