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

For 20 years, clinical reports and laboratory observations have suggested that allogeneic blood transfusion effects important changes in the recipient's immune response. The seminal clinical studies involved dose-dependent improvement in renal allograft survival in patients transfused with allogeneic whole blood, red blood cells, and buffy coat preparations. Subsequently, a burgeoning, but unclear literature proposed that allogeneic blood transfusion decreases survival or tumor-free survival of patients who undergo operations for a variety of different malignancies. Similar studies suggest that the risk of postoperative infection increases when patients receive allogeneic blood. Transfusion reportedly improves some patients with Crohn's disease. In summary, these findings have been interpreted as evidence for an immunosuppressive effect of allogeneic blood transfusion. A small prospective study showed that paternal buffy coat infusion decreases the rate of fetal loss in a subset of women with recurrent abortion. These data suggest induction of "tolerance." Laboratory studies confirm changes in lymphocyte subsets, lymphocyte activation, natural killer cell activity, antigen-presenting function, and phagocytic cell function in patients and animals that receive allogeneic blood. The clinical relevance of these observations remains controversial. Allogeneic leukocytes induce expression of latent cell-associated viruses (human immunodeficiency virus, cytomegalovirus, Epstein-Barr virus), suggesting further immune-mediated adverse effects of transfusion. The mechanisms and clinical importance of these observations have become areas of intense interest and investigation for transfusion medicine.
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PMID:Immunologic aspects of blood transfusion. 820 21

A mannoprotein fraction (MP-F2: mannan, > 90%; protein, 4.5%) from the human commensal microorganism Candida albicans was as efficient as interleukin-2 (IL-2) in generating cytotoxicity against the uninfected or human immunodeficiency virus type-1 (HIV-1) persistently infected monocytoid U937 cell line in cultured peripheral blood mononuclear cells (PBMC) from healthy human subjects. MP-F2-activated killing of U937 cells (U937-MAK) decreased progressively with advancing stages of HIV-1 infection to virtually no killing effect in PBMC from advanced AIDS subjects (AIDS PBMC). This decrease paralleled a lowered susceptibility of U937 cells to natural killer cell activity. In contrast, IL-2-activated killing of U937 cells (U937-LAK) was not affected by the progression of HIV infection and persisted at high levels in AIDS PBMC. To shed light on the mechanisms of U937-MAK and its decrease during HIV infection, IL-1 beta, IL-6, TNF-alpha, GM-CSF, and IFN-gamma production was analyzed. Decreases in TNF-alpha, GM-CSF, and IFN-gamma, but not IL-1 beta or IL-6, levels were observed in MP-F2-stimulated PBMC from HIV-infected subjects, compared to healthy controls. Interestingly, these cytokine levels fell before the onset of AIDS. The greatest relative drop was that of IFN-gamma, from 4600 (+/- 600) to 290 (+/- 160) and 217 (+/- 110) mean pg/ml (+/- SE) in PBMC from healthy donors (11 subjects), CDC stages II + III (14 subjects), and CDC stage IV (10 subjects), respectively. The following observations suggest that decreased IFN-gamma production plays a role in the abrogation of U937-MAK activity: (i) addition of neutralizing anti-IFN-gamma antibodies abolished both IFN-gamma and U937-MAK activity in PBMC from healthy subjects; (ii) substantial levels of IFN-gamma were detected in supernatants of PBMC cultures stimulated by IL-2, in line with preserved U937-LAK activity. Interestingly, anti-IFN-gamma antibodies also abolished TNF-alpha production, and the anti-TNF-alpha antiserum effect was comparable to that of anti-IFN-gamma in U937-MAK inhibition. In contrast, anti-TNF-alpha antibodies abrogated TNF-alpha activity, but only partially reduced IFN-gamma production. Thus, in human PBMC, U937-MAK activity progressively decreases with advancing stages of HIV infection, whereas U937-LAK activity is sustained. Furthermore, the present results indicate a pivotal role for IFN-gamma in U937 MAK activity, possibly through activation of TNF-alpha production.
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PMID:Mannoprotein-induced anti-U937 cell cytotoxicity in peripheral blood mononuclear cells from uninfected or HIV-infected subjects: role of interferon-gamma and tumor necrosis factor-alpha. 825 54

The env gene of human immunodeficiency virus type 1 (HIV-1) encodes gp 120/41 which plays an important role in the viral infection process and pathogenesis. The surface glycoprotein gp120 is a candidate molecule for the development of a subunit vaccine against HIV-1-induced acquired immunodeficiency syndrome (AIDS). However, thorough studies on the immunobiology of this molecule are hampered by the lack of a suitable model. With this background in mind, and in order to learn more on anti-gp120 cellular immunity, we attempted to develop gp120-expressing human cell clones. Thus by transfecting a human lymphoid cell line of B lineage (Raji), which is known to be resistant to the natural killer cell activity, with an expression vector encoding the envelope and vpu, we established three clones that stably express gp120/41 and vpu. The surface glycoprotein gp 120 is also expressed on the cell surface of these clones. The transfected cells from syncytia with CD4+ human cell lines as well as with peripheral blood mononuclear cells (PBMC) leading to the death of the fused cells. This observation represents additional evidence for the eventual depletion of CD4+ viral targets that fuse with adjacent HIV-infected, gp 120-expressing cells. The latent Epstein-Barr virus genome present in the transfected cells, was not induced to express the lytic cycle antigens. The densities of the surface expression of a number of molecules examined remained unchanged in the transfected cells except for the surface IgM, which increased significantly (P < 0.05) in two clones. One of the clones exhibited a significantly (P < 0.05) reduced proliferation rate as compared to the other clones. The transfected cells of all the three clones showed a significantly (P < 0.01) increased susceptibility to lysis by the PBMC from normal, healthy individuals in a 16-hr 51Cr-release assay. This is the first report of the MHC- and antibody-independent lysis of human cells transfected with the HIV-1 surface glycoprotein. The transfected cells also served as targets in a gp120-specific antibody-dependent cellular cytotoxicity assay. We anticipate that the present model will prove very useful for studying the gp120-specific immune responses in HIV-infected individuals.
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PMID:Stable expression of the transfected HIV-1 env gene in a human B cell line: characterization of gp120-expressing clones and immunobiological studies. 842 93

Optimal conditions for in vitro anti-human immunodeficiency virus type 1 (HIV-1) antibody (Ab) synthesis and detection were re-appraised. Western blot (WB) and radioimmunoassay (RIA) could detect about 1 and 10 ng, respectively, of HIV-1-specific Ab (HIV-Ab), while the sensitivity of an enzyme-linked immunosorbent assay (ELISA) was much lower. Optimal HIV-Ab recovery was obtained by culturing 2.5 x 10(6) peripheral blood mononuclear cells (PBMC)/ml from seropositive subjects for 16 days in the absence of mitogens; at higher cell concentrations, background levels were unacceptably high. The background of non-de novo synthesized HIV-Ab was due to insufficient PBMC washing and/or cytophilic immunoglobulin (Ig); a particular washing procedure, as well as 24 h peripheral blood mononuclear cells (PBMC) pre-culture, might help in limiting this phenomenon. However, results should be compared with those obtained in cultures containing puromycin especially in infants, where a higher CD16 antigen expression in lymphocytes is likely responsible for increased amounts of cytophilic Ig released in culture supernatants, compared to adults.
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PMID:Standardization of in vitro synthesis and detection of HIV-1-specific antibodies. 842 52

Yesterday's immunodeficiencies emphasized the clinical and familial associations of the syndromes and date from the 1920s (ataxia-telangiectasia, chronic mucocutaneous candidiasis), the 1930s (Wiskott-Aldrich syndrome), skipping the 1940s, but blossoming in the 15-y period from 1950 to 1965. In this period, primary immunodeficiencies affecting all the major limbs of the immune system were first described (1950: severe combined immunodeficiency; 1952: X-linked agammaglobulinemia; 1957: chronic granulomatous disease; 1965: C2 deficiency). Today's immunodeficiencies, as detailed in Stiehm's Immunologic Disorders in Infants and Children (Edition 1, 1973; Edition 2, 1980; and Edition 3, 1989) emphasize the immunologic and genetic aspects of immunodeficiency. These increased from 43 syndromes in the 1973 edition (34 primary, nine secondary) to 94 syndromes in the 1989 edition (66 primary, 28 secondary). This means that about two primary and one secondary immunodeficiencies have been uncovered annually. Tomorrow's immunodeficiencies, to be covered in Edition 4, will include new clinical and immunologic observations and molecular and biochemical studies that characterize some unique immunodeficiencies. These include the following six groups of defects: 1) neutropenic syndromes with hypogammaglobulinemia, including the WHIM syndrome; 2) phenotypic genetic syndromes with immunodeficiency including Bloom's syndrome and Schimke's immuno-osseous dysplasia; 3) natural killer cell defects associated with a) other primary immunodeficiencies, b) other nonimmunologic illness, and c) primary natural killer defects; 4) T-cell membrane defects; 5) IL defects; and 6) miscellaneous phagocytic illnesses including periodontitis and the asplenia syndrome.
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PMID:New and old immunodeficiencies. 843 70

A 6 month old boy was diagnosed as a case of combined immunodeficiency (with predominant T cell defect by previous classification). His T cell count was decreased, his B cell count in peripheral blood was increased, his serum IgG level was decreased, his serum IgM level was normal and the thymus was not evident on CT scans and magnetic resonance imaging. Administration of the thymus hormone, thymosin, led to a partial recovery of T cell function without normalization of the T cell count. At age 26 months the patient received an irradiated thymus transplantation from a 16 week old female fetus. After the transplantation, the T cell count (mainly CD4+ cells) increased by 50-70%. A mild graft-versus-host reaction (GVHR) occurred and several immunosuppressants were prescribed. Chromosome analysis showed that the T cells have both 46 XY and 46 XX karyotypes while the B cells have the 46 XY karyotype alone. His cellular immunity (skin tests, DNA synthesis, mixed lymphocyte reaction, cytotoxic activity and natural killer cell function) and his serum IgG level remained low. However, being on regular r-globulin therapy and oral anti-fungal drugs, he is now living normally with almost no trouble at age 6 years and 3 months. This case showed that irradiated thymus transplantation might be a useful method when an adequate donor for bone marrow transplantation is not available. The unexpected observation that the increased T cells were mainly CD4 may be related to the mild GVHR and the clinical improvement.
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PMID:Irradiated fetal thymus transplantation in a patient with combined immunodeficiency with predominant T cell defect. 846 May 43

Growth hormone-releasing hormone (GHRH), growth hormone (GH), prolactin (PRL) and insulin-like growth factor I (IGF-I) are synthesized and secreted by various immunocompetent cells. In addition, GHRH, GH, PRL and IGF-I receptors are expressed on immune cells. Growth hormone, PRL and IGF-I stimulate the proliferation of immunocompetent cells and modulate humoral and cellular immune functions, i.e. immunoglobuline secretion of B cells, thymulin secretion of thymic epithelial cells, natural killer cell activity, phagocytosis, oxidative burst and killing capacity of neutrophils and macrophages. No clinically significant cellular or humoral immunodeficiency has been found in GH-deficient patients. However, several immunological parameters and functions are altered in GH-deficient patients when compared to normal controls. The data available to date indicate that endocrine and pleiotropic para- and autocrine mechanisms of action are involved in a neuropeptide immune network, including GH, PRL and IGF-I as modulators of immune function.
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PMID:Effects of growth hormone and insulin-like growth factor I on the immune system. 854 46

The DiGeorge syndrome has been associated with various immune deficits. Embryologically, defects of the neural crest are associated with conotruncal and aortic arch abnormalities. The objective of this study was to determine if children with neural crest congenital heart defects can have subtle but significant immunodeficiencies. Complete blood counts with differential counts and a standard lymphocyte immunophenotyping panel of selected monoclonal antibodies were performed on peripheral blood from 20 children with neural crest cardiac disease and 34 normal newborns. The children with cardiac disease were grouped as survivors and nonsurvivors. The mean total white blood cell count was similar for all groups, but the percent lymphocytes was significantly less in the nonsurvivors than in the survivors and normal newborns (p < 0. 02). The lymphocyte subsets affected were CD2, CD3, and CD4. When the cardiac patients were compared to the normal newborns, again differences in lymphocyte subsets CD2, CD3, and CD4 were seen. When comparing nonsurvivors with survivors, the mean percentages of the CD2, CD3, and CD4 T lymphocyte markers, as well as the mean lymphocyte, B cell (CD20), and natural killer cell (CD16) percentages were all lower in the nonsurvivors. It was concluded that abnormalities in specific lymphocyte populations and their subsets may be predictors of infants at greatest risk for immunodeficiency complications. Therefore children with neural crest cardiac defects should have evaluations of lymphocyte subsets at birth and be treated as if potentially immunodeficient.
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PMID:Abnormalities in lymphocyte populations in infants with neural crest cardiovascular defects. 866 26

Measles is often fatal for immunocompromised hosts. Protective immunity against measles has been studied but is still not completely understood. Recently, five cases of measles were encountered in immunocompromised children. Two of these were allogeneic bone marrow transplanted cases (one common variable immunodeficiency and one severe aplastic anemia) in remission, one Wilms' tumor case in remission, one hepatoblastoma case after cytotoxic therapy at disease onset and one exaggerating hemophagocytic syndrome case with suppressed natural killer cell activity. Clinical symptoms, laboratory findings and the immunologic backgrounds of these five patients were investigated. One of the patients, an 8 year old boy with hemophagocytic syndrome, died of giant cell pneumonia which was confirmed in the section of necropsy lung specimen. Two other patients who received allogeneic bone marrow transplants were not immune to measles, despite their own and their donors' immunizations. Their clinical symptoms were rather severe but both patients recovered and have remained seropositive for as long as 13 months. This fatality from measles is the first reported in a patient with hemophagocytic syndrome. Suppressed natural killer cell activity may be a poor prognostic factor. Also, secondary immunization failure for measles can occur in bone marrow transplanted patients with rather severe clinical symptoms.
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PMID:Clinical features of measles in immunocompromised children. 874 8

The levels of prostaglandins in human semen are many orders of magnitude higher than those found elsewhere in the body and semen contains 19-hydroxy PGE which has not been found in other tissues. The reason for the presence of these prostaglandins is now becoming apparent with the demonstration of powerful effects of PGE and 19-hydroxy PGE on the balance of cytokines (stimulating IL-10 and inhibiting IL-12) released by antigen presenting cells. The effects of the seminal prostaglandins will be two-fold. First, there will be cAMP mediated direct effect on T cells, inhibiting clonal proliferation, inhibiting natural killer cell function and biasing the CD4 cells to a T-helper-2 pattern of cytokine production away from one that would favour a cell-mediated response. Second, and perhaps the major effect, is at the level of the antigen presenting cell that will reinforce the direct effects and induce a tolerance of antigens that are presented together with the IL-10, or PGE. Such tolerance might be necessary for the survival of the spermatozoa under adverse conditions, for instance, in the presence of infection Viruses and other invading organisms would also benefit from this switch in cytokines and the inhibition of the cell-mediated defences. Particular concerns are human immunodeficiency virus (HIV) and human papilloma virus (HPV) which can be transmitted in semen. Not only will the initial immune response be affected, but also repeated exposure to semen will reduce immunesurveillance and the removal of virally infected cells.
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PMID:Prostaglandins in primate semen: biasing the immune system to benefit spermatozoa and virus? 925 Jun 93


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