Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A large inbred family is described in which there were seven cases of Hodgkin's disease, three of lymphosarcoma, two of thymoma, two of common variable immunodeficiency, and single cases of retinoblastoma, neuroblastoma, and rhabdomyosarcoma. There have been no other lymphoma cases in the community during the past decade. Further study of this family may help to define the genetic basis for development of Hodgkin's disease and other disorders.
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PMID:Common variable immunodeficiency, Hodgkin's disease, and other malignancies in a Newfoundland family. 4 22

Leukocyte migration inhibitory factor (LMIF) production in mixed lymphocyte culture (MLC) reactions is the result of cellular interactions based on two separate phenomena: the capacity of lymphocytes to stimulate in MLC, and the capacity of lymphocytes to respond in MLC. Puromycin-treated lymphocytes are capable of stimulating allogeneic cells for LMIF production, but are unable to respond with synthesis of LMIF (one-way MLC-LMIF test). We have studied the stimulating and responding capacity of lymphocytes from patients with different immunodeficiency syndromes in a one-way MLC-LMIF assay. Lymphocytes from patients known to have qualitative and quantitative defects of T cell or B cell functions (Hodgkin's disease, mycosis fungoides, thymoma, chronic lymphatic leukemia) were found to respond poorly as measured by mediator production although their stimulating fuction was frequently retained. Patients with advanced solid tumors often had both MLC-stimulating and responding functions depressed. There was no apparent correlation between mitogen response and MLC-induced LMIF responses or between MLC proliferative response (as measured by thymidine incorporation) and mediator production. Studying of stimulatory and responding capacity of lymphocytes in the MLC-LMIF assay provides a new tool for assessing immunocompetence and allows for in vitro evaluation of cellular interactions that may play an important role in vivo.
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PMID:Human immunodeficiency disease: impairment of cellular interactions leading to abnormal mediator production in mixed lymphocyte culture reaction. 13 47

Immunoglobulin (Ig) synthesis and secretion by peripheral blood lymphocytes activated by pokeweed mitogen (PWM), and by phytohemagglutinin (PHA) was measured in normal individuals and in subjects with primary immunodeficiency. Unstimulated cultures demonstrated stable, low Ig synthesis of all major Ig classes; PWM showed a peak of Ig synthesis at day 5; PHA cultures demonstrated a late peak of ig production at day 9. Three cases of common variable immunodeficiency showed different patterns of data when the percentage of B cells in blood and Ig production in vitro were used as parameters. Two persons with immunodeficiency and thymoma showed decreased Ig production in vitro, and had suppressor cells capable of blocking Ig production by normal lymphocytes in co-cultivation experments.
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PMID:Differentiation of human lymphocytes by pokeweed mitogen in vitro: studies in normal and in immunodeficient subjects. 30 69

T lymphocytes with receptors for IgM(Tmu) and IgG(Tgamma) were examined in thirty patients with primary immunodeficiency and autoimmune disorders. Six out of twenty-seven patients with primary immunodeficiency had a low proportion of Tmu cells when compared with normal controls. Eight out of twenty-seven patients with primary immunodeficiency had an increased proportion of Tgamma cells. Two out of twenty-seven patients had both a low proportion of Tmu cells and a high proportion of Tgamma cells. The patient studied with severe combined immunodeficiency had a low proportion of both Tmu and Tgamma cells. Patients with Bruton-type agammaglobulinaemia, common variable immunodeficiency, thymoma and immunodeficiency syndrome and selective IgA deficiency demonstrated heterogeneity with regard to alterations in T-cell subpopulations.
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PMID:Subpopulations of human T lymphocytes. I. Studies in immunodeficient patients. 30 82

Blood mononuclear cells of two individuals having immunodeficiency with thymoma (ID-THY) were cocultured with normal mononuclear cells or treated mononuclear cell fractions in an attempt to correct an imbalance of regulatory cells postulated to be responsible for the failure of pokeweed mitogen-induced Ig synthesis in vitro. Treatment included abrogation of suppressor cell activity by irradiation or incubation with prednisilone in vitro. T cell help was provided by cocultivating lymphocytes of related and unrelated persons, and in some cases autologous treated cells. Ig secretion failed to be induced by any experimental maneuver suggesting that the primary problem in the above ID-THY cells was related to defective or deficient B cells rather than an imbalance of T regulatory cells. Prednisilone treatment in vitro decreased suppressor cell activity in allogeneic cocultures of two ID-THY persons (S1 and S2) but not of an individual (S3) with variable immunodeficiency suggesting heterogeneity of suppressor cells.
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PMID:Immunodeficiency with thymoma: failure to induce Ig production in immunodeficient lymphocytes cocultured with normal T cells. 31 Aug 51

The clinical, pathologic and immunologic features of 27 patients with chronic mucocutaneous candidiasis and thymic tumors are reviewed. This form of chronic candidiasis is unique in that the infections do not occur until after the third decade and, in contrast to patients in whom candidiasis develops during infancy or childhood, it is not accompanied by failure of endocrine organs. Instead, the patients have the disorders that often accompany thymoma, such as myasthenia gravis, hypogammaglobulinemia, and abnormalities of the bone marrow and circulating blood elements. Evidence of impaired cell-mediated immunity was found in 16 of the 21 patients in whom studies were made. The pathogenesis of the immunodeficiency in these patients is unknown. Immunosuppressive activities in the plasma of four patients were found, but none of the five patients in whom the appropriate studies were made was found to have suppressor cells. The features of this disorder are unique enough that it should be considered a syndrome, and patients in whom candidiasis develops during their adult years should be studied for the presence of thymoma.
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PMID:Mucocutaneous candidiasis and thymoma. 37 63

Rosette formation with mouse erythrocytes and other cell-surface markers were examined on lymphocytes from patients with a variety of primary immunodeficiency and lymphoproliferative disorders. Mouse erythrocyte rosette-forming cells and lymphocytes with surface immunoglobulins were regularly absent in patients with Bruton type agammaglobulinaemia, immunodeficiency and thymoma syndrome and severe combined immunodeficiency disease. However, they were present in normal or low numbers in patients with common variable immunodeficiency, selective IgA deficiency and ataxis telangiectasia. Lymphocytes from patients with acute lymphoblastic leukaemia Sezary syndrome and mycosis fungoides made no or few rosettes with mouse erythrocytes. Increased numbers of mouse erythrocyte rosette-forming cells were present in patients with chronic lymphocytic leukaemia and Waldenstrom's macroglobulinaemia. The significance of the mouse erythrocyte rosette as a B-cell marker in the analysis of primary immunodeficiency and lymphoproliferative disorders is discussed.
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PMID:Rosette formation with mouse erythrocytes. III. Studies in patients with primary immunodeficiency and lymphoproliferative disorders. 106 59

An 8-year-old male underwent excision of an encapsulated thymoma. Four months later he presented with disseminated, fatal varicella. Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma). This is the first case of Good's syndrome reported in a child and the first case of fatal varicella associated with Good's syndrome. The combination of this rare pediatric tumor and immunodeficiency is discussed. Despite specific antiviral therapy, varicella remains a deadly disease in the immunocompromised host.
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PMID:Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome). 232 70

A 63 yo female was hospitalized with a 2 week history of vomiting, epigastric pain, anorexia and weight loss. She had an incidental finding of left anterior upper mediastinal mass on Chest Xray and TSP of 2.2 gm% and globulins of 1.2 gm% along with endobronchial larvae of Ascaris lumbricoides and malignant cells and Strongyloides stercoralis in the gastric mucosa. She died after a progressively deteriorating course and at autopsy a thymoma predominantly composed of spindle shaped cells was found. This rare variant of thymoma has been associated with red cell agenesis and with immunodeficiency (Good's syndrome).
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PMID:[Thymoma of fusiform cells. Report of a case]. 276 92

A 69-year-old woman sought treatment with a history of persistent debilitating intraoral ulcerations and recurrent oral and vaginal candidiasis. The medical history included thymoma, breast cancer, and lip cancer. The oral lesions were consistent with bullous lichen planus. Laboratory studies showed severe hypogammaglobulinemia. The chronic mucocutaneous candidiasis-thymoma syndrome is now recognized as a distinct form of primary immunodeficiency.
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PMID:Chronic mucocutaneous candidiasis-thymoma syndrome. A case report. 279 38


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