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 13,001 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Individuals with either primary or secondary immunodeficiencies are at high risk to develop not only infections but also malignancy (especially of the lymphoid system). The major focus of this paper is on malignancies that develop in immunodeficiency syndromes, particularly malignancies in naturally occurring immunodeficiencies and following bone marrow transplantation (BMT). As of August, 1986, 514 cases of naturally occurring immunodeficiencies have been registered at the Immunodeficiency Cancer Registry. Overall non-Hodgkin's lymphomas predominate in these patients, accounting for 48.6% of all cases. Non-Hodgkin's lymphoma is the predominant malignancy in ataxia-telangiectasia, common variable immunodeficiency, Wiskott-Aldrich syndrome (WAS) and severe combined immunodeficiency (SCID). The histopathology of the lymphomas differs somewhat in each of the disorders. In WAS, large cell "histiocytic" lymphoma predominates, with most cases having the features of B lymphocytes, including pleomorphic immunocytoma and immunoblastic lymphoma. Non-Hodgkin's lymphoma in SCID also generally has B cell features and in some cases multiple copies of Epstein-Barr virus (EBV) genomic DNA have been found in tumor tissue. In contrast to ataxia-telangiectasia, in which non-Hodgkin's lymphoma is also the predominant neoplasm, the morphology and cell marker characteristics are more similar to those seen in nonimmunodeficient children. The lymphomas in ataxia-telangiectasia are very heterogeneous with representation from all the major histologic subtypes. We have found no relationship between the degree of immunodeficiency and the development of malignancy. Immunodeficiency following BMT, as in naturally occurring immunodeficiencies, appears to predispose patients to the development of lymphoid malignancy, especially for recipients of partially mismatched bone marrow. In Minnesota 8 patients have developed B cell lympho-proliferative disorders (BLPD) following BMT.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Relationship of immunodeficiency to lymphoid malignancy. 284 Jun 29

To date, the acquired immunodeficiency syndrome (AIDS) has been identified in over 50 children in the US, including those with associated hemophilia, high-risk environmental factors (Haitian background, parental intravenous drug abuse, or prostitution), and blood transfusions. The evaluation of an infant or young child in whom AIDS is suspected requires exclusion of congenital disorders of immune function. A specific test is not currently available, but inclusion criteria for childhood AIDS have been developed. The diseases accepted as indicative of underlying cellular immunodeficiency children are the same as those used in defining AIDS in adults, with the exclusion of congenital infections such as toxoplasmosis or herpes simplex virus infection in the 1st month of life or cytomegalovirus infection in the 1st 6 months of life. Specific conditions that must be excluded in children are primary immunodeficiency diseases (e.g., DiGeorge syndrome, Wiskott-Aldrich syndrome, ataxia-telangiectasia, neutrophil function abnormality) and secondary immuno-deficiency associated with immunosuppressive therapy, lymphoreticular malignancy, or starvation. Almost all young children with AIDS have hepatosplenomegaly, interstitial pneumonitis, and poor growth. The average age of 36 US child AIDS victims studied in detail was 5 months at presentation with findings suggestive of severe immunodeficiency. Mucocutaneous candidiasis was present in 75% of these 36 children, and Pneumocystis carinii and cytomegalovirus were each isolated from 30% of cases. Normal T4:T8 ratios occur in about 15% of pediatric AIDS cases. Laboratory evidence of polyclonal hypergammaglobulinemia generally supports the AIDS diagnosis. Recurrent infection and malnutrition are major problems in the clinical management of child AIDS patients.
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PMID:Acquired immune deficiency syndrome in childhood. 298 8

Lymphomas occurred in 3 of 16 Japanese patients with ataxia telangiectasia (AT) and Wiskott-Aldrich syndrome (WAS). The patients had a persistently reactivated Epstein-Barr virus (EBV) infection with a remarkable decrease in virus-specific cellular immunity. In these patients, the B lymphocytes were more sensitive to EBV-induced events and to cellular proto-oncogene activation than seen in the healthy counterparts. This immunologic and genetic background was considered to explain the massive lymphoproliferation in these primary immunodeficiency disorders.
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PMID:Epstein-Barr virus infection and oncogenesis in primary immunodeficiency. 303 Mar 41

Twenty-two cases out of a total of 683 patients (3.2%) with primary immunodeficiency diseases registered in the All-Japan Immunodeficiency Registry were reported to have developed malignant diseases. In the childhood patients with ataxia-telangiectasia the incidence of death due to malignancy was approximately 625 times higher than that of the normal Japanese childhood population. The incidence of lymphoproliferative disorders, such as malignant lymphoma, in Chediak-Higashi syndrome and the incidence of non-Hodgkin lymphoma and various carcinomas in ataxia-telangiectasia were both high, 37.5% and 13.7%, respectively. Only one case out of 45 with Wiskott-Aldrich syndrome was reported to have malignant lymphoma. The data obtained were compared with international statistics reported by the Immunodeficiency Cancer Registry.
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PMID:Primary immunodeficiency diseases and malignancy in Japan. 308 18

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

The peripheral blood leukocytes of 6 children with clinical data suggestive of primary cellular immunodeficiencies were studied in an attempt to define the cellular basis of these disorders. The phenotype and function of T and B cells were investigated. According to the clinical and laboratory features, the patients were classified as one case of severe combined immunodeficiency (SCID), two of ataxia-telangiectasia (AT), one of Wiskott-Aldrich syndrome (WAS), one of DiGeorge syndrome (DSG), and one of cellular immunodeficiency (CID). The laboratory investigations together with the clinical manifestations permitted a diagnosis of primary immunodeficiency diseases.
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PMID:Primary immunodeficiency diseases: a presentation of 6 cases. 326 46

Skin fibroblasts were cultured from 15 patients with primary immunodeficiency diseases associated with a high cancer risk, including sex-linked agammaglobulinemia, IgA deficiency, variable immunodeficiency, ataxia-telangiectasia (cerebellar malfunction and abnormalities of blood vessels and immune response), Wiskott-Aldrich syndrome (low platelet count, eczema, and abnormal immune mechanism), and severe combined system (cellular and humoral) immunodeficiency. Fourteen of 15 cell strains were found to have low or regular susceptibility to transformation with the tumor virus, simian virus 40. The data are consistent with the view that the frequent occurrence of malignancy in patients with primary immunodeficiency is due to abnormalities of the immunologic surveillance mechanism.
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PMID:Susceptibility of cells from patients with primary immunodeficiency diseases to transformation by simian virus 40. 411 42

For thr purpose of clarifing the role of cultured thymic epithelium (CTE) and dialyzable leukocyte extracts (DLE) in the maturation process of T cells, the effects of the thymic epithelial supernatant (TES) and DLE on cyclic nucleotides in thymocytes were studied. TES increased cAMP levels significantly in thymocytes of mice. The activity of TES to increase cAMP levels correlated well with the state of the growth of thymic epithelium. Moreover, TES increased cAMP levels in human thymocytes, and augumented lymphocyte transformation (LT) to mitogens in immunodeficiency diseases. From these effects, it was suggested that TES had the activities such as thymic hormones. CTE of which TES increased cAMP levels in thymocytes of mice were transplanted in patients with Wiskott-Aldrich syndrome and Ataxia-telangiectasia. After the transplantation, augumentation of LT was observed in both patients. From these results, it was speculated that CTE were engrafted and became to exert its effect in the host. We concluded that it was possible to select the CTE appropriately for transplantation by means of examining the activity of TES. The basal levels of human thymocytes were very low compared with those of peripheral blood lymphocytes (PBL). A significant increase of cAMP levels was observed in thymocytes with stimulation of DLE. DLE produced no significant change of cyclic nucleotide levels in PBL. These results suggested that DLE affected the maturation of human thymocytes with involvement of cAMP. Though DLE was proved to contain histamin and prostaglandin E2, it was revealed from the present study that the active component responsible to increase cAMP levels in human thymocytes was different from these substances. Fractions III and IV of DLE obtained with gel filtration showed the activity to increase cAMP. It was suggested that these fractions contained the active component.
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PMID:[The role of cultured thymic epithelium and dialyzable leukocyte extracts on the maturation process of T cell. Study of their effects on cyclic nucleotides levels in thymocytes]. 608 79

Peripheral blood mononuclear cells (PBMC) from 40 patients with a variety of primary immunodeficiency diseases were examined for concanavalin A (Con A) inducible suppressor activity against proliferative response of autologous and allogeneic PBMC to phytohaemagglutinin (PHA). 45% (12/27) of the patients with common variable immunodeficiency and 86% (6/7) of the patients with selective IgA deficiency demonstrated lack of Con A-induced suppressor activity against proliferative response of autologous/allogeneic PBMC. 2 of 4 patients with X-linked agammaglobulin and both patients, each with Wiskott-Aldrich syndrome and ataxia-telangiectasia, also showed deficient suppressor function. This study demonstrates a deficiency of Con A-inducible suppressor-cell activity in a variety of immunodeficiency diseases. Possible underlying mechanisms for this functional defects are discussed.
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PMID:Deficiency of concanavalin A induced suppressor cell activity in patients with primary immunodeficiency disorders. 622 64

Patients with various immunodeficiency and autoimmune disorders were examined for the proportions of T cells bearing Fc-IgA, Fc-IgG, and Fc-IgM receptors. These disorders included common variable immunodeficiency, systemic lupus erythematosus, ataxia-telangiectasia, selective IgA deficiency, and the Wiskott-Aldrich syndrome. Abnormal proportions (higher or lower than an interval containing 95% of our normal values) of Fc-IgA receptor-bearing T cells (TA) were found in common variable immunodeficiency, systemic lupus erythematous, ataxia-telangiectasia, and selective IgA deficiency. However, the proportion of T cells bearing Fc receptors did not correlate with their functional activity in in vitro immunoglobulin production assays.
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PMID:The proportion of TA, TG, and TM cells in various immunodeficiency and autoimmune disorders. 622 12


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