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

The association of immunodeficiency with head and neck squamous cell carcinoma has generated the concept of supplying immunologically active agents as a means of treating these cancers. One of the most active immunologic messengers is interferon gamma, which has been observed in our laboratories to also have a direct cytotoxic effect on cultures of squamous cell carcinoma derived from the head and neck. To test the feasibility of treating patients with advanced but resectable head and neck cancer with this agent, we designed a phase I-II trial of recombinant human interferon gamma using a 24-hour infusion repeated weekly for four times. In this study, both tumor and immunologic parameters were studied before and after treatment. Eight patients were entered into the study with the highest recombinant human interferon gamma dose attempted being 0.25 mg/m2 per 24 hours. Minimal side effects were observed. Three patients had clinically measurable responses, four had stabilization of disease, and one had progression while receiving treatment. Histopathologic results of treatment were similar to in vitro observations. Necrosis, as well as differentiation of tumor cells, was observed. In some tumors there was a marked decrease in cellularity without a change in tumor volume due to increased extracellular keratin deposition. Our study indicates that evaluation of adoptive immunotherapy trials in head and neck cancer needs to include parameters other than simple tumor regression as an end point, otherwise therapeutically important lymphokine-induced changes may be missed. Further evaluation of recombinant human interferon gamma and agents that induce human interferon gamma are warranted.
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PMID:Phase I-II study of advanced head and neck squamous cell carcinoma patients treated with recombinant human interferon gamma. 212 9

As an approach to dissect complex mechanisms that lead to graft-vs-host (GvH)-associated immune disorders, we have compared the splenic cytotoxic T lymphocyte (CTL) response and thymic hormonal function in nonirradiated F1 hybrid mice injected with parental spleen cells. Thymic secretory function was studied by the determination of serum thymulin levels, and the number of thymic epithelial cells containing thymulin as assessed by indirect immunofluorescence with the use of an anti-thymulin monoclonal antibody. In addition, the epithelial cell network was analyzed with an anti-keratin serum, and the general histology pattern was studied by conventional histologic methods. An initial analysis was performed on day 15, which was characterized by CTL suppression mediated by parental suppressor T cells. No thymic abnormalities were detected at this time. By day 45 after GvH induction, active CTL suppression had decreased, and GvH was associated with a progressive decline in thymic hormonal function. Finally, by day 60 and thereafter, F1 GvH mice recovered normal in vitro CTL responsiveness, which contrasted with profound alterations of the epithelial cell network and severely reduced serum thymulin levels. This hormonal dysfunction was shown to be directly associated with a reduction in the number of thymulin-containing cells. Moreover, no anti-thymulin auto-antibodies could be detected. The results are discussed with respect to the role of thymic hormonal dysfunction in the modulation of F1 CTL responses observed during the course of a GvH reaction, and the additional analogy of this GvH model with human immunodeficiency.
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PMID:Cytotoxic T cell response and thymic hormonal dysfunction in graft-vs-host mice. 241 20

Chicken anaemia agent (CAA) causes severe anaemia, loss of body weight, and hypoplasia of thymus at day 14 after inoculation of one-day-old chickens. Several reports have described an enhancement of concurrent infections with f.e. Marek's disease virus, Infectious Bursal Disease virus, and Reovirus. Immunohistochemical methods were used to describe the immunopathological lesions of the thymus that probably form the basis of the immunodeficiency caused by CAA. Monoclonal antibodies and antisera against leucocytes, T lymphocytes, CD4, B lymphocytes, mononuclear phagocytes, MHC class II, and keratin were used. At day 14 after inoculation, the thymic cortex was completely depleted of thymocytes, whereas the medulla was not. T-cell areas in the spleen also lacked T lymphocytes. In contrast the cortex still contained stromal cells with MHC class II molecules and keratin. At day 21, the cortex had completely regenerated and all clinical signs of CAA infection had disappeared. Labelling experiments with BrdU in 4-week-old control chickens demonstrated that 25% of the divided cells was detected in the medulla and 75% in the cortex. The tissue tropism of CAA may, apart from the preference for rapidly dividing cells, be directed by specific cell determinants.
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PMID:Transient depletion of cortical thymocytes induced by chicken anaemia agent. 256 Feb 70

Oral hairy leukoplakia (HL) is a newly described lesion (1984) in human immunodeficiency virus infected patients. Patients with HL show a high probability of developing an acquired immunodeficiency syndrome (AIDS). The results of histopathological, microbiological, immunological and ultrastructural studies in five patients with HL and AIDS are reported. The histopathological exam revealed in all cases acanthosis, parakeratosis, koilocytosis and keratin projections on the surface. Dermis was normal. Herpes type virus were seen in four cases and in one of them papilloma virus was also present in electron microscopic examination. There was immunocytochemical evidence of papilloma virus in one lesion. Candida albicans was found in 5 lesions by culture but only in two ones by periodic acid Schiff stain. Virus cultures for herpes virus were negative. Immunocytochemical search of Langerhans cells (S-100, HLA-DR, OKT4, OKT6) showed nearly absence of these cells in HL lesions. These results favour the viral etiology hypothesis of hairy leukoplakia.
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PMID:[Oral hairy leukoplakia. A study of 5 cases]. 269 22

The epithelial cells of the thymus produce hormones that have been implicated in the maturation of T lymphocytes. Thymic epithelial cells can be encapsulated in a permselective polymer membrane that allows the passage of thymic hormones, but prevents the entry of antibodies, complement, cells, and viruses. A pure culture of thymic epithelial cells, identified as such by transmission electron microscopy and keratin staining, was obtained by low temperature organ culture of fetal mouse thymus. These cells remained intact and produced physiologic amounts of the thymic hormone thymosin alpha-1 after encapsulation in a permselective polymer membrane and in vitro culture for 5 days. An encapsulated implant of thymic epithelial cells may therefore promote reconstitution of an immune system in immunodeficiency diseases without allowing the rejection or destruction of the thymic tissue.
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PMID:Encapsulated thymic epithelial cells as a potential treatment for immunodeficiencies. 319 84

We report a case of atrichia with papular lesions in association with common variable immunodeficiency in an 11-year-old boy. There were identical findings in the patient's father. This rare variant of ectodermal dysplasia typically presents with shedding of normal fetal hair within the first three months. Eyelashes are typically spared. Numerous keratin-filled follicular cysts develop over extensive areas of the skin, usually between the age of 2 and 26 years. These lesions are most numerous on the face, neck, scalp, and extremities. Teeth and nails are entirely normal in these patients. The mode of inheritance of atrichia with papular lesions is uncertain. Our case suggests an autosomal-dominant inheritance pattern, while previous reports have shown autosomal recessive inheritance pattern.
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PMID:Atrichia with papular lesions. 370 74

Thymic biopsies from two patients with combined immunodeficiency and defective expression of HLA class I and class II antigens on blood mononuclear cells ("bare lymphocyte" syndrome) were investigated. This made possible an evaluation of the significance of HLA antigen expression in a detailed (immuno)histologic study. Both thymuses showed a normal lobular architecture with distinct cortex-medulla areas, well-differentiated epithelium, including ultrastructurally defined subtypes, and Hassall's corpuscles. Normal numbers of lymphoid cells were present and normal T-cell phenotype was found. Using anti-HLA-A,B,C antisera, confluent staining of the medulla (stroma and lymphocytes) was observed. One of the thymuses was found to be negative for HLA class II antigen expression: the other revealed only HLA-DR positivity of nonlymphoid cells in the medulla. These cells were not of epithelial nature as judged from double staining with anti-keratin antibody. There was no expression of HLA-DC/DS. These observations differ from findings in the normal thymus, wherein epithelial cells in the cortex carry HLA class I and class II antigens, and epithelial cells in the medulla express HLA class I, and for a minor part class II antigens. The results indicate a normal sequential acquisition of T-cell differentiation antigens in the thymus of both cases. It is suggested that the expression of HLA class I and class II antigens on epithelial cells in the normal thymus cortex does not play a significant role in the sequential acquisition of differentiation antigens on T lymphocytes.
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PMID:The thymus in "bare lymphocyte" syndrome: significance of expression of major histocompatibility complex antigens on thymic epithelial cells in intrathymic T-cell maturation. 387 94

Speculation has existed for decades on the association between the lack of male circumcision and the sexual transmission of disease. It has been suggested that the surface epithelium of the glans develops a protective keratin layer following circumcision which functions like a natural condom against contracting disease. Circumcised males may therefore be less susceptible to contracting sexually transmitted diseases (STD), including HIV. The identification of simian immunodeficiency virus-infected mononuclear cells in the dermis and epidermis of the penile foreskin of macaques also suggests that male circumcision may reduce the infectivity of men with HIV. The authors review the evidence in support of the association between the lack of circumcision and STDs, and the possible biological explanations. They also discuss the implications for public health interventions and suggest areas and methods for further research. Twenty-three published study reports linking circumcision status to HIV infection are identified and include retrospective studies including partner studies, cross-sectional serosurveys, a longitudinal study, and ecological correlations. Five studies linked the lack of circumcision to STDs other than HIV infection. In interpreting the data, the authors consider susceptibility versus infectivity, assessment of behaviors and adjustment for confounding, selection bias, misclassification of exposure, measure of association, and publication bias. It is ultimately concluded that more studies are needed to quantify the relative risk associated with the lack of male circumcision. Observational designs could be employed to that end along with laboratory and primate research.
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PMID:Male circumcision: a role in HIV prevention? 804 24

Primary effusion lymphoma (PEL) has been recognized as a body-cavity-based lymphoma that was originally reported to be associated with human herpes virus 8 (HHV8) infection, and was frequently found in human immunodeficiency virus-positive (HIV) patients. Here we describe an autopsy case of PEL of the peritoneal cavity in an immunocompetent patient. Cytological analysis of tumor cells within ascites revealed immunocytochemical features of keratin positivity and CD45 negativity. At autopsy, the presence of a massive volume of ascites as well as diffuse tumor cell infiltrates within the serosa of the intestine and mesenterium were observed. Tumor cells were morphologically similar to anaplastic large-cell lymphoma, but were immunohistochemically positive for keratin and epithelial membrane antigen (EMA). They also showed no reactivity to representative lymphocyte surface markers including CD45, in addition to being negative for CD30 and p80NPM/ALK. Molecular analysis of the tumor cells revealed monoclonality of the immunoglobulin heavy-chain gene rearrangement which demonstrated a lymphoma of the B-cell lineage. Furthermore, HHV8 was not detected by immunohistochemical analysis, PCR or nested PCR technique. Based on these results, we consider the present case to be an HHV8-negative PEL with keratin and EMA positivity.
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PMID:HHV8-negative primary effusion lymphoma of the peritoneal cavity presenting with a distinct immunohistochemical phenotype. 1135 Jun 13

Omenn syndrome is a severe primary immunodeficiency with putative autoimmune manifestations of the skin and gastrointestinal tract. The disease is caused by hypomorphic mutations in recombination-activating genes that impair but do not abolish the process of VDJ recombination, leading to the generation of autoreactive T cells with a highly restricted receptor repertoire. Loss of central tolerance in genetically determined autoimmune diseases, e.g., autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy, is associated with defective expression by medullary thymic epithelial cells of AIRE, the transcription activator that induces thymic expression of tissue-specific antigens. Analysis of AIRE expression in the thymi of 2 Omenn syndrome patients and 1 SCID patient, by real-time RT-PCR and immunohistochemistry, demonstrated a profound reduction in the levels of AIRE mRNA and protein in patients as compared with a normal control subject. Lack of AIRE was associated with normal or even increased levels of keratin and lymphotoxin-beta receptor mRNAs, while mRNAs of the self-antigens insulin, cytochrome P450 1a2, and fatty acid-binding protein were undetectable in thymi from immunodeficiency patients. These results demonstrate that deficiency of AIRE expression is observed in severe immunodeficiencies characterized by abnormal T cell development and suggest that in Omenn syndrome, the few residual T cell clones that develop may escape negative selection and thereafter expand in the periphery, causing massive autoimmune reactions.
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PMID:AIRE deficiency in thymus of 2 patients with Omenn syndrome. 1569 98


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