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Characterization of common variable immunodeficiency: identification of a
subset of patients with distinctive immunophenotypic and clinical features
[see comments]
JJ Wright, DK Wagner, RM Blaese, C Hagengruber, TA Waldmann and TA Fleisher
Metabolism Branch, National Cancer Institute, National Institutes of
Health, Bethesda, MD 20892.
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.
Volume 76,
Issue 10,
pp. 2046-2051,
11/15/1990
Copyright © 1990 by The American Society of Hematology
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