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Blood, 1 March 2002, Vol. 99, No. 5, pp. 1544-1551
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Severe deficiency of switched memory B cells
(CD27+IgM IgD) in subgroups of
patients with common variable immunodeficiency: a new approach to
classify a heterogeneous disease
Klaus Warnatz,
Axel Denz,
Ruth Dräger,
Moritz Braun,
Christoph Groth,
Guido Wolff-Vorbeck,
Hermann Eibel,
Michael Schlesier, and
Hans Hartmut Peter
From the Division of Rheumatology and Clinical
Immunology, Department of Medicine, and from the Department of Surgery,
University Hospital of Freiburg, Germany.
Hypogammaglobulinemia is the hallmark of common variable
immunodeficiency (CVID) syndrome, a heterogeneous disorder predisposing patients to recurrent bacterial infections. In this study, we investigated the peripheral B-cell compartment of 30 well-characterized CVID patients in comparison to 22 healthy controls. Flow
cytometric analysis of peripheral blood lymphocytes revealed a
reduction of class-switched
CD27+IgM IgD memory B cells
below 0.4% in 77% of our patients (group I), while this B-cell
subpopulation exceeded 0.5% in all healthy donors and in 23% of CVID
patients (group II). These results correlate well with the capacity of
peripheral blood lymphocytes to produce immunoglobulins in vitro upon
stimulation with Staphylococcus aureus Cowan I (SAC)
plus interleukin-2 because the production of immunoglobulin G in vitro
is entirely dependent on the presence of switched memory B cells. The
subdivision of group I into patients with an increased proportion of
CD21 peripheral B cells (> 20%; group Ia) and patients
with normal percentages of CD21 B cells (< 20%; group
Ib) revealed a significant clustering of patients with splenomegaly and
autoimmune cytopenias in group Ia. Based on these observations, we
propose a fast and reliable new classification for CVID patients by
flow cytometric quantification of class-switched memory and immature B
cells in the peripheral blood of patients. Our results point toward
defects at various stages of B-cell differentiation in CVID subgroups
and support the value of a B-cell-oriented classification principle. A
consensus on this new classification system will hopefully provide a
tool for rapidly defining homogeneous subgroups of CVID for functional studies and genetic linkage analysis.
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