LONG-TERM FOLLOW-OF B-CELL SUBPOPULATIONS IN PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY (CVID)

Author(s):  
Jiri Litzman
2005 ◽  
Vol 140 (3) ◽  
pp. 532-539 ◽  
Author(s):  
B. L. Ferry ◽  
J. Jones ◽  
E. A. Bateman ◽  
N. Woodham ◽  
K. Warnatz ◽  
...  

Blood ◽  
2008 ◽  
Vol 111 (1) ◽  
pp. 77-85 ◽  
Author(s):  
Claudia Wehr ◽  
Teemu Kivioja ◽  
Christian Schmitt ◽  
Berne Ferry ◽  
Torsten Witte ◽  
...  

The heterogeneity of common variable immunodeficiency (CVID) calls for a classification addressing pathogenic mechanisms as well as clinical relevance. This European multicenter trial was initiated to develop a consensus of 2 existing classification schemes based on flowcytometric B-cell phenotyping and the clinical course. The clinical evaluation of 303 patients with the established diagnosis of CVID demonstrated a significant coincidence of granulomatous disease, autoimmune cytopenia, and splenomegaly. Phenotyping of B-cell subpopulations confirmed a severe reduction of switched memory B cells in most of the patients that was associated with a higher risk for splenomegaly and granulomatous disease. An expansion of CD21low B cells marked patients with splenomegaly. Lymphadenopathy was significantly linked with transitional B-cell expansion. Based on these findings and pathogenic consideration of B-cell differentiation, we suggest an improved classification for CVID (EUROclass), separating patients with nearly absent B cells (less than 1%), severely reduced switched memory B cells (less than 2%), and expansion of transitional (more than 9%) or CD21low B cells (more than 10%). Whereas the first group contains all patients with severe defects of early B-cell differentiation, severely reduced switched memory B cells indicate a defective germinal center development as found in inducible constimulator (ICOS) or CD40L deficiency. The underlying defects of expanded transitional or CD21low B cells remain to be elucidated. This trial is re-gistered at http://www.uniklinik-freiburg.de/zks/live/uklregister/Oeffentlich.html as UKF000308.


2012 ◽  
Vol 5 ◽  
pp. S189
Author(s):  
Amanda Seba ◽  
Norma de Paula Motta Rubini ◽  
Albertina Varandas Capelo ◽  
Eliane Miranda da Silva ◽  
Marilza Campos de Magalhães ◽  
...  

2013 ◽  
Vol 171 (2) ◽  
pp. 195-200 ◽  
Author(s):  
P. Bright ◽  
S. Grigoriadou ◽  
P. Kamperidis ◽  
M. Buckland ◽  
A. Hickey ◽  
...  

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