Clinical and immunological assessment of 94 patients with primary
humoral immunodeficiency: Common variable immunodeficiency, selective IgA
deficiency and polysaccharide antibody deficiency syndrome
Affiliations: Department of Immunology at Children's Hospital
Ricardo Gutiérrez, Buenos Aires, Argentina
Note: [] Correspondence: Dr. Liliana Bezrodnik, Department of Immunology,
Children's Hospital Ricardo Gutiérrez, Buenos Aires, Argentina. Tel.:
+54 11 4785 9844; Fax: +54 11 4785 9844; E-mail: lbezrodnik@intramed.net.ar,
lbezrodnik@yahoo.com.ar
Abstract: We present the clinical and B cell immunophenotypical
characterization of 94 patients with Common Variable immunodeficiency (CVID),
selective IgA deficiency (SIgAD) and polysaccharide antibody deficiency
syndrome (SAD). Study design: We retrospectively investigated clinical findings
and B cell compartment in 31 patients with CVID, 35 with SIgAD and 28 with SAD.
Regardless of underlying disease, a delay was observed between age at diagnosis
and onset of first symptoms. The predominant clinical findings were upper and
lower respiratory tract infections. Allergic symptoms were more frequent in SAD
and SIgAD patients, hematological and autoimmune manifestations in CVID and
celiac disease in SIgAD. B-cell Immunophenotype abnormalities were observed in
SAD and CVID patients: both had reduced memory B cells
(CD19^{+} CD27^{+}), and increased
transitional B cells (CD24^{ + + } CD38^{+
+}) was found in SAD. We did not find any statistically significant
abnormalities in any of differentiation stages of B cells in SIgAD. Defects of
the B cell compartment were associated with bronchiectasis, splenomegaly,
autoimmunity and/or malignancy in CVID and SAD patients.We conclude that flow
cytometric evaluation of the B cell compartment could be a useful tool for the
diagnosis and follow up of these patients.
Keywords: Common variable immunodeficiency, IgA deficiency, polysaccharide antibody deficiency syndrome
