Characterization of common variable immunodeficiency: identification of a subset of patients with distinctive immunophenotypic and clinical features.

Abstract:

:The peripheral blood lymphocyte surface markers and clinical features of 38 patients with common variable immunodeficiency (CVID) were assessed. These studies identified a subset of CVID consisting of 14 of the 38 patients with a distinctive T-cell immunophenotype and clinical findings. The phenotypic changes were characterized by an abnormally low CD4/CD8 ratio (less than or equal to 0.9) due primarily to a significant increase in CD8 T cells. In addition, there was an expansion in CD8 T cells coexpressing CD57 and increased expression of the activation markers HLA-DR and interleukin-2 receptor (IL-2R) by these cells. This group of immunophenotypically abnormal CVID patients also had characteristic clinical features, including splenomegaly (P less than .02) and in vivo T-cell dysfunction based on the evaluation of delayed-type hypersensitivity (P less than .05). Approximately 71% of these patients had splenomegaly and 42% were anergic in contrast to the remaining group of CVID patients, in which 29% had splenomegaly and 7% were anergic. These findings define a subgroup of CVID patients that have specific immunophenotypic features and functional T-cell abnormalities.

journal_name

Blood

journal_title

Blood

authors

Wright JJ,Wagner DK,Blaese RM,Hagengruber C,Waldmann TA,Fleisher TA

subject

Has Abstract

pub_date

1990-11-15 00:00:00

pages

2046-51

issue

10

eissn

0006-4971

issn

1528-0020

journal_volume

76

pub_type

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