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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty five patients affected by primary immunodeficiency diseases were treated with a polyethylenglicol treated intravenous gammaglobulin. Number of infusions administered during a period of 665 months/patients, was 110. In those cases previously treated with intramuscular gammaglobulin, serum IgG levels were higher after intravenous gammaglobulin administration. Therapeutic response was favourable in most cases. Intravenous gammaglobulin was well tolerated, noticing only two serious and two mild adverse reactions. Patients that suffered adverse reactions with intramuscular gammaglobulin tolerated well intravenous preparation used, except for one patient that after two years in treatment developed IgE mediated antibodies against IgA. Need of an individualized dosage is emphasized.
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PMID:[Treatment of primary immunodeficiencies with intravenous gamma globulin]. 245 92

Diffuse dermatitis and markedly elevated serum IgE concentrations were observed in three adult males who were seropositive for human immunodeficiency virus (HIV) antibody. The clinical features in common for these patients included 1) an adult onset of greater than 6 weeks' duration associated with pruritus, 2) T-helper (CD-4) cell depletion, 3) the lack of overt atopic disease, and 4) the lack of opportunistic infection (except oral thrush) and neoplasia. The mean serum IgE concentration was 5,959 (range: 4,930-6,260) IU/ml. Cutaneous involvement consisted of hyperpigmented papules with variable excoriations and lichenification. Zidovudine was administered to all 3 patients and was associated with cutaneous improvement. Serum IgE concentrations from 19 AIDS patients without cutaneous disease did not show significant elevations. These observations suggest that certain patients with HIV infection can manifest a unique hyper-IgE syndrome associated with diffuse cutaneous disease.
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PMID:Chronic diffuse dermatitis and hyper-IgE in HIV infection. 246 86

Peripheral blood mononuclear cells of human immunodeficiency virus infected patients were cultured in the presence or absence of 10 micrograms/ml human IgE, and culture filtrates were fractionated on IgE-Sepharose. IgE-binding factors were assessed in the acid eluate fraction from IgE-Sepharose by both rosette inhibition assay and radioimmunoassay. Among 22 cases studied, mononuclear cells from 9 patients formed IgE-binding factors in the absence of IgE, and those of 13 cases formed the factors upon incubation with IgE. The major cell source of IgE-binding factors was T cells. In 2 cases of human immunodeficiency virus infection, Fc epsilon receptors were detected on both Leu-2+ and Leu-3+ T cells.
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PMID:Formation of IgE-binding factors by lymphocytes of HIV-infected patients. 252 52

In this report we present the leukocyte phenotypic analysis of 64 cases of primary immune deficiencies (PID). Functional studies related to lymphocyte activation (CD25 (Tac) antigen expression and response to exogenous IL2) as well as immunoregulatory pathways (spontaneous suppressor activities and suppression by soluble factors) were also considered taking immunodeficiency with hyper-IgM (IDHM) as model. The study of mononuclear cell populations with monoclonal antibodies allowed the characterization of defined phenotypes. In common variable immunodeficiency, B cells were present in normal percentages. In sex-linked agammaglobulinemia there was a lack of B lymphocytes and normal distribution of regulatory populations. These results point out the difference between these two entities despite their clinical and infective similarities. Excess of cells expressing CD38 antigen (NV: 4 +/- 2) were found in: predominantly cell mediated immunodeficiency (PCMI): 38 +/- 20; ataxia telangiectasia: 25 +/- 8, hyper-IgE syndrome: 24 +/- 13; Di George syndrome (DGS): 24 +/- 9, chronic mucocutaneous candidiasis: 15 +/- 7. The increased expression of this antigen was correlated with the presence of compromised cellular immunity. The DGS presented the lowest level of CD8 cells (6 +/- 5; NV: 21 +/- 7). In two patients with IDHM, the phenotypic profile was similar to that found in PCMI (low CD3 cells, low CD4/CD8 ratio and elevated CD38 cells). The depressed proliferative response to PHA demonstrates a cellular immune defect. In both patients we found a low expression of CD25 antigen in stimulated cells. Moreover, the addition of exogenous IL2 decreased the proliferative response to PHA in a dose-dependent fashion, suggesting that the cells expressing the CD25 antigen have suppressor capacity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Lymphocytic, phenotypic and functional studies in primary immunodeficiencies]. 264 Apr 82

Figure 1 depicts some of the potential interactions of the interleukins. Among the substances discussed here, only IL-2 has been used to any large degree in a clinical series. Other cytokines not discussed including some of the colony stimulating factors, tumor necrosis factor and the interferons have also been used in clinical trials. Undoubtedly as we learn more about interleukins IL-1 through IL-7, clinical applications will become apparent. For the allergist/immunologist there are two areas of greatest potential interest. The first of these is in treating immunodeficiency states. Preliminary studies of the use of IL-2 in patients with T cell dysfunction suggest that this substance may be useful in treating selective T cell disorders. IL-4, 5, and 6 all have some influence on B cell function. It is likely that in the near future one or more of these agents will be used clinically. It is also clear that the interleukins have the potential to influence basic mechanisms known to be important in allergic disease. IL-3 is the major factor influencing mast cell growth. IL-4 among other things, promotes B cells to switch to IgE synthesis as well as to induce Fc epsilon RII receptors on B cells. IL-5 is important in the differentiation and growth of eosinophils. Finally, IL-6 is the terminal differentiation factor that causes B cells to become plasma cells. The next few years should result in an even better understanding of the role of each of these interleukins. It is likely that such information will greatly expand the horizons for understanding the pathogenesis of many immunologically mediated diseases and will provide the basis for new modalities of treatment.
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PMID:Interleukins in immunologic and allergic diseases. 267 43

The use of tolerogenic conjugates of monomethoxypolyethylene glycol (mPEG) and diverse antigens, including xenogeneic monoclonal immunoglobulins, is reviewed with respect to the induction of specific abrogation of antibody responses. Evidence is presented for activation of specific suppressor T (Ts) cells by antigen-mPEG conjugates and for the release of suppressor lymphokines (TsFs) from these cells; both Ts cells and TsFs are able to suppress antigen-specific immune responses on transfer to syngeneic mice. The therapeutic potential of tolerogenic mPEG conjugates is discussed with respect to IgE-mediated allergies and diseases which could be ameliorated by administration of xenogeneic monoclonal antibodies and of their respective immunotoxins, as would be the case for suppression of rejection of organ transplants, localization and destruction of tumors, and in vivo annihilation of the human immunodeficiency virus (HIV).
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PMID:Modulation of antibody responses by conjugates of antigens with monomethoxypolyethylene glycol. 269 31

Levels of IgE protein and IgE antibodies specific for 8 different allergenic extracts were measured in the serum of a large series of patients infected by the human immunodeficiency virus (HIV) and in HIV-seronegative subjects belonging to the same risk groups (intravenous drug-users, homosexual men and hemophiliacs). The proportion of subjects showing elevated IgE levels was higher among HIV-infected patients with group IV disease than among HIV-infected patients with group II-III diseases or seronegative individuals. In addition, many HIV-infected patients with elevated IgE levels showed the presence in their serum of IgE antibodies specific for fungal antigens.
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PMID:Increased production of IgE protein and IgE antibodies specific for fungal antigens in patients with the acquired immunodeficiency syndrome. 276 30

We report a case of Burkitt's lymphoma developing in a 7-year-old boy with hyper-IgE syndrome. This is the third reported case of malignancy in the hyper-IgE syndrome. The other two cases were an 18-year-old man with Hodgkin's disease and a 10-year-old girl with histiocytic lymphoma. The patient developed retroperitoneal Burkitt's lymphoma with probable metastasis to the brain. His short life was characterized by recurrent staphylococcal skin, middle ear, and lung infections associated with extremely elevated serum concentrations of IgE. There was also an associated disturbance of bone metabolism with osteoporosis and pathologic fractures and absence of parathormone, findings that have been observed in other patients with hyper-IgE syndrome and other forms of T cell immunodeficiency. At the age of 5 years, inadequate B cell responses to immunization with antigens derived from diphtheria, tetanus, and Haemophilus influenzae type b organisms and with the OX174 bacteriophage were demonstrated in the patient. In his terminal state his in vitro lymphocyte analysis demonstrated findings of anergy. Although the precise immunologic defect in hyper-IgE syndrome is unknown, these cases of associated malignancy stress the role that a completely normal immune system plays in preventing the premature appearance of cancer.
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PMID:Burkitt's lymphoma developing in a 7-year-old boy with hyper-IgE syndrome. 278 97

The expression of Fc epsilon R on human lymphocytes was studied with the anti-Fc epsilon R mAbs. Fc epsilon R was expressed on most mu+,delta+ circulating B cells, whereas T cells did not express Fc epsilon R even in patients with hyper-IgE syndrome. B cells with gamma, alpha, or epsilon phenotype did not express Fc epsilon R, moreover its expression could not be induced, suggesting that the Fc epsilon R expression was correlated with isotype switching. mu+delta+ B cells in bone marrow did not express Fc epsilon R, but PHA-sup (supernatant from PHA-stimulated cell cultures) could induce its expression, and the addition of IgE augmented this induction. Recombinant IL-2, IL-1, IFN-gamma or -beta, or purified B cell differentiation factor (BSF-2 B cell-stimulatory factor 2) could not induce Fc epsilon R expression in bone marrow B cells. IFN-gamma inhibited the Fc epsilon R expression induced by PHA-sup, suggesting that the human counterpart of BSF-1 may be responsible for Fc epsilon R expression in bone marrow B cells. B cells from patients with common variable immunodeficiency and ataxia telangiectasia did not express Fc epsilon R, but PHA-sup could induce its expression, indicating that circulating B cells of these patients are at a differentiation stage similar to B cells in bone marrow. The study showed that Fc epsilon R is a B cell-specific differentiation marker, the expression of which is restricted to a defined stage of B cell differentiation.
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PMID:Fc epsilon receptor, a specific differentiation marker transiently expressed on mature B cells before isotype switching. 294 90

We have selected 11 patients with primary immunodeficiency disorders predominantly affecting T lymphocyte function (four with ataxia-telangiectasia (AT), four with common variable immunodeficiency (CVI) and one each with Wiskott-Aldrich syndrome, hyper-IgE syndrome and combined immunodeficiency) with defective gamma interferon (IFN-gamma) production in vitro. Induction with phytohaemagglutinin showed low interleukin 2 (IL-2) production concomitant with reduced IFN-gamma titres. However the addition of 10 U/ml of rIL-2 to cultures stimulated with staphylococcal enterotoxin B or galactose oxidase failed to restore IFN-gamma production in defective cases. IFN-gamma was titrated by both bioassay and immunoradiometric assay, ruling out the possible release of inactive or altered IFN-gamma molecules. Normal levels of IFN-gamma were found in patients of patients with AT, as well as in two AT and two CVI cases, demonstrating heterogeneity of defects within these syndromes. Soluble inhibitors or cellular suppression of IFN-gamma were not observed in mixing experiments. The possibility that defective interaction between accessory cells and T lymphocytes might account for the poor response to the inducing agents was ruled out as no IFN-gamma was produced using a calcium ionophore--which bypasses this step--in seven patients with absolute IFN-gamma deficiency.
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PMID:Evidence that defective gamma interferon production in patients with primary immunodeficiencies is due to intrinsic incompetence of lymphocytes. 313 28


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