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PubMed1992

Genetic and immunologic analysis of a family containing five patients with common-variable immune deficiency or selective IgA deficiency.

A family with 13 members included 2 subjects with selective IgA deficiency (IgA-D) and 3 subjects with common-variable immune deficiency (CVID), diseases which usually occur sporadically. Reciprocal combinations of B and T cells in vitro between one normal and two immune-deficient family members and normal subjects revealed that defective Ig synthesis was determined by the B cells, while the patient T cells functioned normally. Normal T helper and suppressor function was demonstrated even in one patient with CVID who developed a T-cell lymphoproliferative disorder associated with elevated IgM; this patient's B cells made only IgM in vitro. Immune deficiencies were inherited in this family in a pattern consistent with an autosomal dominant trait with incomplete penetrance. All the immune-deficient patients in this family possessed at least one copy of an MHC haplotype previously shown to be abnormally frequent in IgA-D and CVID: HLA-DQB1*0201, HLA-DR3, C4B-Sf, C4A-deleted, G11-15, Bf-0.4, C2-a, HSP70-7.5, TNF alpha-5, HLA-B8, and HLA-A1. The patient who developed the lymphoproliferative disorder was homozygous for this haplotype. Four immunologically normal members, one of whom was 80 years old, also possessed this MHC haplotype, indicating that its presence is not sufficient for disease expression. A small segment of another MHC haplotype associated with Ig deficiency in the population also occurred in this family, but it was not associated with immune deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1999

Discordance between IgA switching at the DNA level and IgA expression at the mRNA level in IgA-deficient patients.

IgA deficiency is a common immune disorder in Caucasians and is associated with certain MHC conserved extended haplotypes, such as [HLA-B8, SC01, DR3], which presumably carry a susceptibility gene(s). We applied a competitive digestion-circularization PCR method to quantitate the number of switch (S)mu to S alpha rearrangements in peripheral B cells from IgA-deficient subjects homozygous for this haplotype and compared their number with the productive C alpha mRNA level to determine C alpha gene expression in IgA-switched B cells. Two types of defects, low expression of both secreted and membrane forms of productive C alpha mRNA in IgA-switched B cells and impaired IgA switching, were characterized in IgA-deficient subjects homozygous for [HLA-B8, SC01, DR3]. The former defect was also found in another noncarrier subject. It may directly cause low IgA secretion and reflects a blockade in post-IgA switch differentiation of B cells. These results suggest that the heterogeneity of defects in IgA deficiency is not simply ascribable to MHC susceptibility genes.

B-Lymphocytes↗
PubMed2000

[IgG subclasses and autoantibodies in adult patients with selective IgA deficiency].

Selective IgA deficiency is the most common primary immunodeficiency disease, but the etiopathogenesis is unknown. In a portion of patients disturbed IgA production is accompanied by various immunological abnormalities. Serological laboratory results of 30 female and 22 male adult patients with selective IgA deficiency were compared with sex- and age-matched healthy controls. Hypergammaglobulinemia IgG was observed in 31 patients and only in 2 control persons. Serum IgG1 was increased in 12 and/or IgG3 in 18 patients, the increase in IgG2 was less common (6 persons). The number of persons with increased IgE did not differ from the control group. The occurrence of autoantibodies (antinuclear antibodies, rheumatoid factor, antithyroglobulin and anti-thyroid microsomal antibodies, anti-gastric parietal cells, reticulin, smooth muscle, anticardiolipin, and anti-gliadin antibodies) did not differ significantly from the control group. IgG hypergammaglobulinemia, which, according to our results, is the most frequent accompanying serological abnormality in IgA deficiency, may be caused by compensatory increased production, but may also reflect more profound immunological dysregulation in the disease.

Adolescent↗
PubMed1981

Transitory and persistent IgA deficiency. Reevaluation of 19 pediatric patients once found to be deficient in serum IGA.

In 21 out of 5181 serum samples tested between 1968 and 1977 IgA was not detectable. In this subsequent study, 19 individuals could be reinvestigated by clinical and laboratory methods. At reevaluation 26% showed high, 16% normal and 58% subnormal serum IgA. Late maturation of the IgA system therefore occurs frequently. Lack of secretory IgA was proven in 16% and the material used were tears: even in the group with subnormal IgA clinical manifestations are minimal. Development, compensatory mechanisms and criteria for a new definition of selective IgA deficiency are discussed.

Child↗
PubMed1996

Development of a common variable immunodeficiency in IgA-deficient patients.

IgA deficiency (IgA-D) and common variable immunodeficiency (CVID) are two primary immunodeficiencies that share clinical features. Occasionally, both diseases have been diagnosed in the same family, which suggests the existence of some common pathogenic mechanism, but progression from IgA-D to CVID has rarely been documented. We report three cases of CVID diagnosed 1 to 12 years after IgA-D was detected. Two of these patients presented autoimmune diseases followed by a progressive decline in IgG levels. They are currently on intravenous immunoglobulin therapy with complete remission of their autoimmune and infectious symptoms.

Adult↗
PubMed1992

Transfusion management of patients with IgA deficiency and anti-IgA during liver transplantation.

Severe anaphylactic or allergic reactions may occur during blood transfusion to patients who are IgA-deficient and have anti-IgA in their blood, particularly those with class-specific antibodies. These patients are a particular challenge to the hospital transfusion service when large volumes of blood components are required for transfusion support, as in liver transplantation. We have successfully provided blood components for 3 such patients undergoing liver transplantation. Red cells were washed manually or by automated technique. Platelets were washed manually. All plasma was from IgA-deficient donors. One patient's entire plasma requirements were supplied by autologous plasmapheresis. Serial determinations of IgA levels and anti-IgA titers in 1 patient demonstrated an abrupt fall in anti-IgA with the appearance of barely detectable amounts of IgA during the surgery. IgA-containing plasma cells were demonstrated in the biopsies of liver homografts of 2 patients following transplantation. IgA deficiency with anti-IgA can be successfully managed during liver transplantation with advance planning.

Adolescent↗
PubMed1995

DNA typing of DQ and DR alleles in IgA-deficient subjects.

IgA deficiency (IgA-D) represents the most common immunodeficiency syndrome of infancy. In most cases IgA-D represents an isolated immunological disorder, while sometimes it is associated with IgG subclass deficiency or with the presence of autoantibodies. We investigated the pattern of association of IgA-D with DRB1 and DQB1 loci of the HLA region by DNA molecular typing, which allows the identification of previously serologically undefined specificities. We also compared the gene frequency of DRB1 and DQB1 allelic variants between IgA-D subjects with or without serum autoantibodies. Our results indicate that the gene frequency of the DRB1*0102 subtype and of the DRB1*0102, DQB1*0501 haplotype is significantly higher in IgA-D than in the general population. Furthermore, the IgA-D subjects with autoantibodies showed a positive association with DR4 and DR13 subtypes, thus supporting the hypothesis that genetic factors are also involved in the association between IgA-D and autoantibodies.

Alleles↗
PubMed1993

Immunoglobulin secreting cells in lymphocytes of patients with IgA deficiency or hyper-IgA.

To assess the ability of immunoglobulin production in vivo, we enumerated the immunoglobulin secreting cells in the peripheral blood of patients with an IgA deficiency and of those with hyper-IgA-emia. All seven patients with primary IgA deficiency and two of the three patients with secondary IgA deficiency had low numbers of IgA secreting cells. In all five patients with hyper-IgA the number of IgA secreting cells was increased. Our results suggest that measurement of immunoglobulin secreting cells in PBMCs is useful in the assessment of ability of immunoglobulin production in vivo.

Antibody-Producing Cells↗
PubMed1986

Selective IgA deficiency.

Selective IgA deficiency is the most common form of immunodeficiency. Certain select populations, including allergic individuals, patients with autoimmune and gastrointestinal tract disease and patients with recurrent upper respiratory tract illnesses, have an increased incidence of this disorder. These patients have the unique ability to form various antibodies and auto-antibodies including anti-IgA antibodies. Failure of terminal differentiation of the B lymphocyte is the primary defect in IgA deficiency. Treatment should be directed toward the underlying disease associated with this immune disorder. These patients should be advised of the consequences of improperly administered blood or blood products.

Antibodies, Anti-Idiotypic↗
PubMed1992

Effects of serial plasmapheresis on serum IgA levels in IgA-deficient blood donors with IgA-suppressor T cells.

Seventeen IgA-deficient blood donors, without antibodies to IgA, underwent plasmapheresis four to eight consecutive times at intervals of 8 weeks or less to provide fresh-frozen plasma for patients with anti-IgA. Blood samples, drawn for analysis no more than 1 hour before plasmapheresis and again at the conclusion of each procedure, were analyzed for lymphocyte subpopulations and serum IgA levels. Five lymphocyte subpopulations, including natural killer cells, the suppressor-inducer CD4 subset, the suppressor-precursor CD8 subset, non-major histocompatibility complex (MHC)-restricted cytotoxic T cells, and CD5+ B cells, were all decreased significantly after plasmapheresis (p less than 0.05). In a subgroup of IgA-deficient donors with excessive IgA-suppressor T-cell activity, serum IgA increased to levels exceeding 0.05 g per L following the fourth consecutive plasmapheresis procedure. Serum IgA levels did not similarly increase in IgA-deficient donors without excessive IgA-suppressor T-cell activity or in controls without IgA deficiency. Our study shows the potential, in a subpopulation of IgA-deficient donors who undergo frequent plasmapheresis, for a transient increase in serum IgA to a level no longer considered IgA deficient.

Antibody Formation↗
PubMed1988

Serum IgA deficiency and anti-IgA antibodies in pernicious anemia.

Three pernicious anemia (PA) patients with selective IgA deficiency and anti-IgA antibodies in their sera were followed for over 3 years. After instituting therapy with cyanocobalamin there was a slight increase in the anti-IgA antibodies. After 1 year the titers of anti-IgA antibodies in the sera of these patients declined significantly as compared to the values before treatment (P less than 0.02), and after 2 years one patient had no measurable anti-IgA antibodies, yet no IgA appeared in the serum of any of the three. Further, in a medium with no anti-IgA the lymphocytes of these patients were not capable of producing IgA in vitro. Thus, the reason for the IgA deficiency in PA appears to be linked to the function of B cells rather than to anti-IgA antibodies.

Adolescent↗
PubMed1976

Virus antibody levels in IgA deficiency.

IgA-deficient blood donors and their age- and sex-matched controls were compared for the occurrence of complement-fixing antibodies in serum against several viruses. The level in the IgA-deficient persons was slightly higher against several respiratory pathogens (adenoviruses, type B influenza virus, parainfluenza virus, and respiratory syncytial virus) that give rise to localized infections, and against coxsackie B group of viruses. No corresponding difference was observed in mumps, varicella, and cytomegalovirus infections, where viraemia is a characteristic feature, or in Mycoplasma pneumoniae infection.

Adenoviruses, Human↗
PubMedDate not supplied

IgA2 allotypes determined by restriction fragment length polymorphism in IgA deficiency. Re-expression of the silent A2m(2) allotype in the children of IgA-deficient patients.

IgA2 allotyping was performed on DNA from 60 IgA-deficient Caucasian individuals. The frequency of A2m(2) was not statistically different from that of normal controls. Two informative families were selected for further studies. In both families, the A2m(2) allotype (derived from the IgA-deficient parent) was inherited by some of the children, as determined by Southern blotting experiments. In all cases the 'silent' IgA2m2 gene was re-expressed, as judged by conventional serological allotyping of serum proteins. These data strongly argue against structural gene deletions or mutations as a cause of IgA deficiency.

Alleles↗
PubMed1994

Interleukin 10 induces B lymphocytes from IgA-deficient patients to secrete IgA.

We have previously shown that human B lymphocytes cultured in the CD40 system, composed of an anti-CD40 mAb presented by a CD32-transfected fibroblastic cell line, proliferate but do not secrete antibodies. However, the addition of particles of Staphylococcus aureus Cowan (SAC) induces B cell differentiation even in the absence of exogenous cytokines (CD40/SAC system). Additionally, B lymphocytes cultured in the CD40 system in the presence of human IL-10, produce IgM, IgG, and IgA, and Ig levels are further increased by SAC. Here, we have studied the capacity of peripheral blood lymphocytes from patients with IgA deficiency (IgA-D) to secrete Igs, particularly IgA after CD40 triggering. Peripheral blood mononuclear cells (PBMNC) from IgA-D patients cultured in the CD40/SAC system produced IgM and IgG, but not IgA. The addition of IL-10 to the cultures, enhanced the production of IgM and IgG and most strikingly induced the production of high amounts of IgA. The addition of IL-10 to PBMNC from IgA-D patients activated through CD40 alone resulted in the production of IgA. Thus, SAC and anti-CD40 mAb stimulate B cells to differentiate into cells secreting IgG and IgM whereas IL-10 plays a central role in inducing B cells from IgA-D patients to differentiate into IgA secreting cells.

Adolescent↗
PubMed2004

Transfusion of apheresis PLTs from IgA-deficient donors with anti-IgA is not associated with an increase in transfusion reactions.

BACKGROUND: Passive transfer of antibodies through transfusion has been reported to cause adverse consequences. Approximately 1 in 700 individuals of Northern European ancestry lack IgA with up to 40 percent reported as having anti-IgA. Whether these antibodies when passively transfused were associated with reactions was examined. STUDY DESIGN AND METHODS: Records were reviewed to identify IgA-deficient (<0.05 mg/dL detectable IgA) apheresis PLT donors with anti-IgA. Transfusion outcomes of products from these donors were compared to outcomes of control transfusions from donors who were not IgA-deficient. RESULTS: Four IgA-deficient donors with anti-IgA donated a total of 25 apheresis PLT products transfused to 22 recipients. Of the transfused products, 1 (4.0%) was associated with fever that was classified as unrelated to transfusion. In the control group, 60 donors donated 78 apheresis PLT products transfused to 56 recipients. Among these, an allergic reaction was identified (1.3%). The reaction rates between both groups were not different whether the single event in the anti-IgA group was considered a reaction (chi-squared value 0.735, p = 0.3914) or considered unrelated to transfusion (chi-square value 0.324, p = 0.5694). CONCLUSION: The transfusion of products containing anti-IgA does not appear to be associated with a greater frequency of reactions.

Adolescent↗
PubMed1976

[Serum IgE in patients suffering from IgA deficiency with or without atopy].

Numerous studies, based upon the frequency of allergic and auto-immune disorders in association with selective deficiency in IgA, as well as the abnormally high proportion of IgA deficiency in atopic patients, have led to the hypothesis that allergy results from a deficiency in IgA during the first months of life. Thus subjects with an initial transient deficiency in IgA (followed by normal or greater than average production) produce an excess of IgE. The aim of this study, carried out by multifactorial data analysis, was to determine whether serum IgE levels were related to those of IgA. Comparison of the serum Ig levels of 4 groups of individuals, with or without IgA deficiency, showed that the production of IgE was more closely related to the respiratory condition responsible than to IgA levels. According to IgA and E levels, the 4 groups differ distinctly, atopic patients without asthma constituting a "bridge" between the control group and those with asthma. In the presence of an apparently identical situation, i.e. an IgA deficiency, individuals may react in two different ways: some produce an abundance of IgE whilst others fail to do so and are more susceptible to recurrent infective episodes. The role of the genetic control of IgE as well as environmental factors in the pathogenesis of allergic manifestations is discussed.

Age Factors↗