Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
5
pubmed:dateCreated
2003-11-6
pubmed:abstractText
Common variable immunodeficiency (CVID) is a very heterogeneous syndrome defined by impaired immunoglobulin production. The functional classification of CVID patients on the basis of in vitro immunoglobulin production is time consuming and has never shown any predictive value. We propose a classification based on the quantitative repartition of naive/memory B cells according to the dual expression of IgD and CD27. Fifty-seven patients were categorized into three groups: Group MB2 (11 patients, 19%) with normal memory B cells; Group MB1 (19 patients, 33%) with defective switched memory (IgD-CD27+) but normal nonswitched memory B cells (IgD+CD27+); Group MB0 (27 patients, 47%) with almost no memory B cells. In addition, a downexpression of activation markers (CD25, CD21, CD80, CD86) on B cells characterized the group MB1 patients and was associated with an upexpression of activation markers (HLA-DR, CD95, CD57) on T cells. This classification correlates with some clinical aspects showing a higher prevalence of splenomegaly (16/27, 59%), lymphoid proliferation (13/27, 48%) and granulomatous disease (12/27, 44%) in group MB0. Splenomegaly was also frequent in group MB1 (8/19, 42%). In contrast, autoimmunity was observed with similar prevalence in all three groups. Moreover, by analyzing B cell phenotype, immunoglobulin transcript expression, and somatic mutations, we propose different putative mechanisms responsible for impaired B cell activation and memory differentiation in this syndrome.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
0271-9142
pubmed:author
pubmed:issnType
Print
pubmed:volume
23
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
385-400
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed-meshheading:14601647-Adult, pubmed-meshheading:14601647-Aged, pubmed-meshheading:14601647-Aged, 80 and over, pubmed-meshheading:14601647-Antigens, CD27, pubmed-meshheading:14601647-B-Lymphocytes, pubmed-meshheading:14601647-Cell Differentiation, pubmed-meshheading:14601647-Cells, Cultured, pubmed-meshheading:14601647-Common Variable Immunodeficiency, pubmed-meshheading:14601647-Female, pubmed-meshheading:14601647-Humans, pubmed-meshheading:14601647-Immunoglobulin Class Switching, pubmed-meshheading:14601647-Immunoglobulin D, pubmed-meshheading:14601647-Immunologic Memory, pubmed-meshheading:14601647-Immunophenotyping, pubmed-meshheading:14601647-Male, pubmed-meshheading:14601647-Middle Aged, pubmed-meshheading:14601647-Somatic Hypermutation, Immunoglobulin, pubmed-meshheading:14601647-T-Lymphocytes
pubmed:year
2003
pubmed:articleTitle
Common variable immunodeficiency patient classification based on impaired B cell memory differentiation correlates with clinical aspects.
pubmed:affiliation
Laboratoire d'Immunotogie Cellulaire, INSERM U543, Hôpital Pitié-Salpétrière, Paris, France. piqueras@infobiogen.fr
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't