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
Beschreibung
Zusammenfassung:Copyright © 2010 Elsevier Inc. All rights reserved.
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
Beschreibung:Date Completed 26.11.2010
Date Revised 20.10.2021
published: Print-Electronic
Citation Status MEDLINE
ISSN:1521-7035
DOI:10.1016/j.clim.2010.08.008