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• W82664027 abstract "Dear Sir,immunoglobulin A (IgA) was first identified in serum about 50 years ago and is the second most abundant serum immunoglobulin after IgG. Shortly afterwards, IgA was found to be the main immunoglobulin in secretions. Although secretory IgA has many known functions, the role of serum IgA remains unclear.Serum IgA deficiency was first described in children with ataxia-telangiectasia, but was later identified in other patients and populations of normal subjects. The prevalence of IgA deficiency reported in blood donors ranges from 1:400 to 1:3000 in Western countries; the prevalence is lower in some Asian countries1. Most affected persons are asymptomatic, but some IgA-deficient subjects suffer from recurrent sino-pulmonary infections, autoimmunity or allergies. IgA deficiency can be transient in children, but in adults the deficiency is usually permanent 1,2.Since its establishment, 42 cases of selective IgA deficiency, mainly in Iranian children, have been reported to the National Registry of Primary Immunodeficiency3; the present study was designed to obtain an insight into the prevalence of IgA deficiency in healthy Iranian blood donors, an issue which has not been previously studied. In general, knowledge about this immunodeficiency in Middle Eastern countries is rather poor.Since Iran is a multi-ethnic country, but with areas of fairly homogenous genetic background, the immunodeficiency was studied in various different regions, as well as in Tehran, the capital city. For this purpose samples were taken from healthy volunteer blood donors from four regional centres of the Iranian Blood Transfusion Organisation after routine physical examination and completion of the required questionnaire and consent form. The first centre, where 3250 samples was collected, was Tehran which was used as an example of a multi-ethnic urban city with a representative general population. The second centre selected, Bandare Turkman, is in the north-east of Iran; the majority of the population here is of Turkman origin and 900 samples from healthy donors were collected. The third centre, Kermanshah, is in the west of Iran with a population consisting mainly of Kurds; 3622 samples were collected in this centre. Finally, 1080 samples from blood donors in the Bushehr centre, in the south of Iran, were collected. The ages of the blood donors ranged from 18–63 years.Another group, consisting of 95 first degree family members of the IgA deficient individuals, was also studied.In order to compare the prevalence determined among the different groups, the chi-square test or Fisher’s exact test was used.Differences were considered statistically significant at p values <0.05.Immunoglobulins were estimated using commercial immunodiffusion plates, Nor-Partigen from Behring (Marburg, Germany). The first screening was carried out using standard plates with a detection limit of 5 mg/dL. Subjects whose samples were found to be deficient in IgA (less than 5 mg/dL) were rechecked using a second sample taken after 3–6 months; in these cases, “low concentration” plates from the same company, with a detection level of less than 1 mg/dL were used. The IgA deficient samples were also checked for levels of IgG and IgM using specific immunodiffusion plates, Nor-Partigen IgG and IgM (Behring, Marburg, Germany).The results obtained from screening the 8852 healthy blood donors in the four regional centres are summarised in table I, which clearly shows that the prevalence of IgA deficiency is significantly different between the various geographical regions. This deficiency was detected in 1:450 subjects in Bandar Turkman, this being a significantly higher prevalence than in the other three regions studied.Table IIgA deficiency in blood donors in four Blood Transfusion Regional centres in IranRepeating the IgA assay with “low concentration” immunodiffusion plates on a second sample taken after 3–6 months from the IgA deficient subjects confirmed the persistence of the IgA deficiency in these healthy adult individuals.The samples from the nine IgA deficient individuals were also tested for IgG and IgM levels; these values were within the normal range in all cases, and no concomitant deficiency was observed.Finally, the incidence of IgA deficiency among 95 first-degree relatives of the immunodeficient individuals was 5%, which is 50 times higher than in unrelated subjects.An important factor when considering the frequency of IgA deficiency is the definition of the deficiency which, in the present study, was the classical definition of <5 mg/dL. However, we found that all the immunodeficient subjects had IgA levels of less than 1mg/dl.In this study of healthy blood donors, the overall incidence of selective IgA deficiency was 1.02/1000. This figure is close to the range reported for Europe and North America; unfortunately there are no reports for Middle Eastern populations for comparison. However, the data presented in this study may be an indication that incidence rate in this part of the world is probably higher than that reported for some Asian countries 1.The significant difference in the incidence observed between various regions within Iran confirmed the possible role of genetic background and probable consanguinity. The incidence observed in the capital city, Tehran, demonstrates that in an open and ethnically mixed population the incidence of IgA deficiency is lower than in more stable, rural and genetically homogeneous populations, in which the probability of consanguinity is higher. Similar differences have been reported for monoclonal gammopathy in different French populations.The incidence of IgA deficiency in the southern region of Iran seems very low and a further study on larger populations is required.IgA deficiency appears to be inherited in sporadically, although familial inheritance in an autosomal recessive and autosomal dominant fashion with variable or incomplete expression has been described. Ethnic variability is also recognised, with higher frequencies in Caucasians and a low frequency in the oriental populations studied1,2; the results of the present study, showing significant differences between various geographic regions in Iran, is further evidence of this genetic variation.When family members (95 cases) of the Iranian IgA deficient individuals were studied, no additional IgA deficient members were discovered in five families. In the remaining families, the prevalence of IgA deficiency was 5%, which is 50 times higher than the unrelated cases and is close to the 7.3% (38-fold increase) reported in some previous genetic studies. As far as the molecular genetics of IgA deficiency is concerned, an association with some translocations in chromosome has been reported.The immune defect found in the IgA deficient blood donors in this study seems not to have interfered with a normal healthy life. The levels of the major immunoglobulins (IgG and IgM) were within normal limits and the histories of the IgA deficient subjects revealed no more infections than those in blood donors without this deficiency.Finally, it should be noted that IgA deficiency is a major concern for blood transfusion services since IgA-deficient individuals exposed to blood products containing IgA may develop anti-IgA antibodies that can cause anaphylactic reactions1. Despite yielding a definitive diagnosis in fewer than 20% of anaphylactic transfusion reactions, investigation for IgA deficiency and the presence of presumably pathogenic IgG anti-IgA is useful in patient management. Individuals with demonstrated anti-IgA must receive IgA-depleted cellular products or IgA-deficient plasma and derivatives to prevent recurrent severe reactions 4. A fatal reaction due to anti-IgA, occurring 45 min after administration of about 50 mL of blood, has been reported; passive transfer of anti-IgA through products containing anti-IgA does not, however, appear to cause a greater frequency of reactions.The scarcity of IgA-deficient banked plasma products and dedicated plateletpheresies donors has led the American Association of Blood Banks to develop policies to allocate such precious resources appropriately5. Developing similar policies in all countries where an average prevalence of IgA deficiency is reported is strongly recommended." @default.
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• W82664027 date "2009-04-01" @default.
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• W82664027 title "Selective deficiency of immunoglobulin A among healthy voluntary blood donors in Iran." @default.
• W82664027 doi "https://doi.org/10.2450/2009.0047-08" @default.
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