Iranian Journal of Allergy, Asthma and Immunology
Volume:4 Issue: 1, Mar 2005

Primary Immunodeficiencies constitute a group of highly complex congenital disorders most of which are characterized by a very poor prognosis. Allogeneic hematopoietic stem cell transplantation (HSCT) has become an established curative treatment approach in many of these disorders, which may be permanently corrected. In this presentation basic and practical aspects of HSCT are presented, with an emphasis on its application in lymphocyte disorders such as severe combined immunodeficiency (SCID). Optimal results and outcome of HSCT are highly dependant on early and correct diagnosis of these rare disorders, and HSCT should usually be applied early in the course of the disease in order to prevent irreversible complications from infections. Clinical results will be summarized based on recent analysis performed in large patient cohorts, which have shown steady improvements and have led to a marked change in the prognosis of patients with primary immunodeficiencies.

Controversial immunomodulatory properties of bee venom (BV) have provided an appropriate field for more investigation. The aim of present research was to verify the effects of honeybee venom on matrix metalloproteinase activity and interferon production as well as cell proliferation in monocyte and fibroblast cell lines.The monocyte and fibroblast cell lines (K562, HT-1080, WEHI-164) were used in order to assess proliferative response, interferon-1 production and matrix metalloproteinase-2 (MMP-2) activity. Australian BV (ABV) and Iranian BV (IBV) preparations at concentrations of 0.025, 0.05, 0.1, 0.2, 0.3, 0.4, 0.5, and 1µg/ml were added to each overnight cultured cells. In time course study, cells were treated each with ABV and IBV. In all cases supernatants were collected 24 hours after treatment. A sample of the each medium was used for zymography and interferons assay. Non-treated cells were used as controls.The production of IFN-a and IFN-b in supernatant of cell cultures was assessed using enzyme linked immunoassay procedure. MMP-2 activity, as an inflammatory index, was evaluated using zymoanalysis method.The results of this study showed that, there were no significant difference between two sources of honey bee venoms when they were added to an identical cell line, whereas, the responses of various cell lines against bee venom were different. The increasing amounts of bee venom to human monocyte cell line (K562) revealed a significant increase in proliferative response. Our findings showed that the bee venom had no influence on IFN-a production in cell culture media, whereas, adding the BV to K562 cell line could significantly increase the production level of IFN-b only on day 8 post-treatment. In addition the effect of bee venom on MMP-2 activity in both cell culture media, WEHI-164 and K562 was similar. The stimulatory effect of bee venom on MMP-2 activity occurred at low doses. In contrast, its inhibitory effect was seen at high concentrations.honeybee venom affects on MMP-2 activity and interferon beta production as well as cell proliferation in a time and dose-dependent manner.

This study investigated the in vitro production of interferon-γ, interleukin (IL)-10, IL-12, and IL-13, after antigenic stimulation of the cells (with Leishmania antigen and lipopolysaccharide) using whole blood from patients with cutaneous leishmaniasis lesions caused by Leishmania tropica and in normal volunteers with history of cutaneous leishmaniasis.ELISA results showed that the mean production of interferon-γ by cells of whole blood in patients with lesions in response to Leishmania antigen was significantly lower than corresponding values in volunteers with history of cutaneous leishmaniasis (P< 0.05) and significantly higher levels of IL-10 production in patients with lesions were observed compared with cured volunteers of the disease (P<0.01). A similar level of IL-12, including p40 subunit of IL-12, was detected in both groups tested in this study in response to stimulation of parasite antigen. The levels of the IL-13 after stimulation with Leishmania antigen were significantly more in patients compared with volunteers with history of cutaneous leishmaniasis (P< 0.01). There was no significant difference in the mean production of IFN-γ, IL-10, IL-12 and IL-13 by PHA or LPS stimulated cells from patients with lesions and volunteers with history of the disease, indicating that there was no qualitative defect in cytokine production in these patients.In this study, we have detected the decreased production of interferon- γ by cells of patients with lesions of cutaneous leishmaniasis in response to parasite antigen and unbalanced production of regulatory cytokines such as IL-10 and IL-13 using the whole-blood stimulation assay technique. The required small volume of blood and the rapid set up time are the advantages in this assay technique. Using this assay for further immunodetection of cytokines may confirm its value for clinical investigation.

Tuberculosis is a chronic mycobacterial infection. The main effector cells against mycobacterium tuberculosis are CD4+ T lymphocytes. Our objective in this research was to evaluate the quantity of T lymphocytes and their subpopulations before and after treatments with combination of 4 drugs (Rifampcin, Isoniaside, pyrasinamide, Ethambutal) for 2 months directly in sputum-positive tuberculosis patients. Twenty patients as cases and twenty healthy people were selected as controls. Flow cytometry was used for TCD3+, TCD4+ and TCD8+ lymphocytes by using monoclonal antibodies. Our results indicated that there was alteration in cell mediated immunity during tuberculosis showing itself as decrease in TCD3+ and TCD4+ lymphocytes and increase in TCD8+ lymphocytes. The changes in TCD3+ and TCD4+ but not in TCD8+ were reversible after 2 months of treatment.

During the last two decades or so, the incidence of fungal infections has increased dramatically. Deep- seated mycoses are creating serious problems for clinicians working with certain populations of patients, such as those with cancer, the immunocompromised, and physiologically compromised.A study of fungal isolated for identification of deep fungal infections, risk factors and etiologic agents in immunocompromised patients was carried out in the section of Medical Mycology, Pasteur Institute of Iran from 1994 to 2001. Seventy one immunosuppressed patients with deep fungal infection were retrospectively analyzed for etiology and risk factors. They had one or more predisposing factors to disseminated fungal infections. Diagnosis was established by demonstration of fungus in direct and cultural examinations. Candida spp. were isolated in 70.4% (39.4% C. albicans and 30.9% non-albincans), and Aspergillus spp. were isolated in 14.1% of cases. The most frequent risk factors were hematologic malignancy (ALL, lymphoma, Hodgkin, multiple myeloma) and diabetes mellitus. This study suggests that in immunocompromised patients, fungal infections especially in saprophytic infections, background evaluation and clinical features, correspondence of clinical symptoms and laboratory examinations should be considered and investigation of other factors which created the infection will lead us to a clear picture of patients situation.

Obesity has been reported to be associated with an increase in asthma in children. If there is any association, it could be attributed to an effect of obesity on lung volume and thus airway’s obstruction. Data from 2413 children aged 7–12 years in Isfahan were analyzed. The subjects were included in this study if data were available for: height, weight, age, lung volume, and any measure of asthma, including history of diagnosed asthma, wheeze, chronic cough, and medication as obtained by questionnaire. Body mass index (BMI) percentiles, divided into quintiles per year age, were used as a measure of standardized weight.After adjusting for, sex, age, smoking and family history, BMI was a significant risk factor for wheeze ever (p = 0.000) and asthma ever (p = 0.000), diagnosed asthma (P=0.000) and current asthma (p = 0.000). There was no significant correlation between BMI and obstructive spirometry. Increased BMI was significantly associated with an increased airway resistance.Despite the fact that higher BMI is a risk factor for, wheeze ever, wheeze and dyspnea in the last 12 months, and diagnosed asthma, higher BMI is not a risk factor for obstructive pattern in pulmonary function test.

Chronic Mucocutaneous Candidiasis (CMCC) refers to a group of immunodeficiencies, characterized by persistent or recurrent infections of the skin, nails, and mucus membranes caused by candida. A wide range of immunologic abnormality has been reported in CMCC. Defects in cellular limb of the immune system, mainly the specific response to antigens of candida species, are well documented in CMCC patients. A subgroup of patients is predisposed to development of autoimmune endocrinopathies. These patients need repeated monitoring of endocrine functions. Immunologic studies are needed to identify the extent of immunodeficiency and other abnormalities of immune functions. We report three cases of CMCC. These patients show different phenotypes and highlight the need for complete evaluation and long term follow-up for accompanying disorders.

CD8 deficiency is a rare primary immunodeficiency with low or absent peripheral CD8 cells which results from TAP deficiency, Zap 70 deficiency and CD8 α gene mutation.We report a 14 year old female who presented with a history of recurrent pneumonia, bronchiectasis, otitis, severe varicella, herpetic lesions of mouth, bilateral uveitis, and cataract formation since the age of 8 years.She had growth failure, a huge spleen and moderate clubbing. In immunologic workup, humoral and phagocytic systems were normal. DTH response to candida, PPD and DT were negative but LTT response to PHA mitogen was normal. HLA typing showed normal class I expression. Flowcytometry of peripheral blood showed CD8: 0 to 2% (absolute count, 0-60 cells/mm3) with increased CD4/CD8 ratio on several occasions.Diagnosis of this patient cannot be HLA class I deficiency (TAP1 or TAP2), because class I expression had been normal. It is possible to be Zap -70 deficiency or CD8 α gene mutation. Bilateral uveitis in our patient was a unique presentation which might have resulted because of immune dysregulation in CID.