Clinical and laboratory aspects of common variable immunodeficiency

Abstract

Common variable immunodeficiency (CVID) is an immunological disorder characterized by defective antibody production, recurrent infections, most notably of the respiratory tract, autoimmune phenomena and cancer. Some CVID patients may also present disturbances of the cellular immune response such as a decrease in the number and proportion of different lymphocyte populations, diminished lymphoproliferative response to mitogens and antigens, altered production of cytokines, and deficient expression of cell-surface molecules. Most Brazilian CVID patients included in this study show a decrease in T and B lymphocyte counts in the peripheral blood. Furthermore, their lymphocytes are more susceptible to apoptosis following activation than normal individuals, and they have a decrease in the expression of activation molecules like CD25, CD69, CD40L and CD70. Moreover, they show a decreased synthesis of IL-4 and IL-5 in comparison with normal individuals. The increase in susceptibility to apoptosis following activation, may also be responsible for the decrease in the expression of activation molecules and CD40L, decrease in Th2 cytokines synthesis, and in the number of T and B circulating cells. In this study we discuss some of these immunological disturbances correlating them to the patients' clinical features and comparing our patients' findings to the literature.

Salivary Immunoglobulins in Individuals with Common Variable Immunodeficiency

“…The primary phenotypic defect is a failure in B cell differentiation, resulting in impaired secretion of IgA, IgG and IgM (2). Diagnosis is difficult because of the infrequent incidence, high clinical variability of CVID and lack of specific laboratory tests, and is generally established years after the onset of the disease (3)(4)(5).…”

Dominant-negative effect of the heterozygous C104R TACI mutation in common variable immunodeficiency (CVID)

“…CVID is characterized by markedly reduced serum levels of IgG, IgA, and often IgM (1). In addition to having low levels of Igs, patients with CVID fail to produce specific antibodies after vaccination or exposure to antigens (2). CVID patients suffer from recurrent sinopulmonary, ear, and gastrointestinal infections and have increased risk of autoimmune and lymphoproliferative diseases (1).…”

A single center 14 years study of infectious complications leading to hospitalization of patients with primary antibody deficiencies

“…; other studies showed that the most frequently identifi ed organisms causing infectious diarrhea were Giardia lamblia, Campylobacter sp., and Salmonella sp. 6,7,25 Although Crypotosporidium parvum is a common pathogen among HIGM patients, 19 it was found in only one patient in our study, which could be due to the small number of these patients in the current series.…”