Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency

Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency

Copyright © 2010 Elsevier Inc. All rights reserved.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 137(2010), 3 vom: 24. Dez., Seite 357-65
1. Verfasser: Rensing-Ehl, A (VerfasserIn)
Weitere Verfasser: 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, M G, Minkov, M, Sirkiä, K, Kopp, M V, Korhonen, M, Schwarz, K, Ehl, S, Speckmann, C
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2010
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Comparative Study Journal Article Research Support, Non-U.S. Gov't Biomarkers Fas Ligand Protein Immunoglobulin G fas Receptor Interleukin-10 130068-27-8 Vitamin B 12 P6YC3EG204
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245 1 0 |a Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency 
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500 |a Date Revised 20.10.2021 
500 |a published: Print-Electronic 
500 |a Citation Status MEDLINE 
520 |a Copyright © 2010 Elsevier Inc. All rights reserved. 
520 |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 
650 4 |a Comparative Study 
650 4 |a Journal Article 
650 4 |a Research Support, Non-U.S. Gov't 
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650 7 |a Immunoglobulin G  |2 NLM 
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650 7 |a 130068-27-8  |2 NLM 
650 7 |a Vitamin B 12  |2 NLM 
650 7 |a P6YC3EG204  |2 NLM 
700 1 |a Warnatz, K  |e verfasserin  |4 aut 
700 1 |a Fuchs, S  |e verfasserin  |4 aut 
700 1 |a Schlesier, M  |e verfasserin  |4 aut 
700 1 |a Salzer, U  |e verfasserin  |4 aut 
700 1 |a Draeger, R  |e verfasserin  |4 aut 
700 1 |a Bondzio, I  |e verfasserin  |4 aut 
700 1 |a Joos, Y  |e verfasserin  |4 aut 
700 1 |a Janda, A  |e verfasserin  |4 aut 
700 1 |a Gomes, M  |e verfasserin  |4 aut 
700 1 |a Abinun, M  |e verfasserin  |4 aut 
700 1 |a Hambleton, S  |e verfasserin  |4 aut 
700 1 |a Cant, A  |e verfasserin  |4 aut 
700 1 |a Shackley, F  |e verfasserin  |4 aut 
700 1 |a Flood, T  |e verfasserin  |4 aut 
700 1 |a Waruiru, C  |e verfasserin  |4 aut 
700 1 |a Beutel, K  |e verfasserin  |4 aut 
700 1 |a Siepermann, K  |e verfasserin  |4 aut 
700 1 |a Dueckers, G  |e verfasserin  |4 aut 
700 1 |a Niehues, T  |e verfasserin  |4 aut 
700 1 |a Wiesel, T  |e verfasserin  |4 aut 
700 1 |a Schuster, V  |e verfasserin  |4 aut 
700 1 |a Seidel, M G  |e verfasserin  |4 aut 
700 1 |a Minkov, M  |e verfasserin  |4 aut 
700 1 |a Sirkiä, K  |e verfasserin  |4 aut 
700 1 |a Kopp, M V  |e verfasserin  |4 aut 
700 1 |a Korhonen, M  |e verfasserin  |4 aut 
700 1 |a Schwarz, K  |e verfasserin  |4 aut 
700 1 |a Ehl, S  |e verfasserin  |4 aut 
700 1 |a Speckmann, C  |e verfasserin  |4 aut 
773 0 8 |i Enthalten in  |t Clinical immunology (Orlando, Fla.)  |d 1999  |g 137(2010), 3 vom: 24. Dez., Seite 357-65  |w (DE-627)NLM098196855  |x 1521-7035  |7 nnns 
773 1 8 |g volume:137  |g year:2010  |g number:3  |g day:24  |g month:12  |g pages:357-65 
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