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At least 775 records · Page 43Linked to original sources
PubMed1980

Subpopulations of human T lymphocytes. XV. T lymphocytes with receptors for IgA (T alpha), a distinct subpopulation of T lymphocytes. Studies in patients with primary immunodeficiency disorders.

A receptor for IgA was observed on a subset of T cells (T alpha) that is distinct from other T lymphocyte subsets, T mu or T gamma cells. IgA receptor on T alpha cells is blocked by IgA from human serum through its cytophilic attachment. Neither T mu nor T gamma cells, following an in vitro interaction with insoluble immune complexes during the process of purification and further incubation at 37 degrees C, changed their phenotypes to T alpha cells. However, some T gamma cells demonstrated transition to T mu cells. The numbers and proportion of T alpha cells in patients with selective IgA deficiency were either normal, increased or decreased. The significance of T alpha cell analysis in thirty-one patients with primary immunodeficiency disorders including those with selective IgA deficiency is discussed.

Antigen-Antibody Complex↗
PubMedDate not supplied

[Incomplete Digeorge syndrome (author's transl)].

A newborn with multiple malformations, recurrent infections and hypocalcemic tetany is presented. The malformations included: Facial asymmetry, micrognathia, epicantus, low set nose, peculiar ears, club-feet and heart disease. The immunologic work-up disclosed a cellular immunodeficiency, but normal humoral immunity, and a diagnosis of "incomplete DiGeorge syndrome" was made. No thymus, nor parathyroid glands were found at autopsy. The disagreement between the analytical data and the pathological findings of "complete DiGeorge syndrome" are discussed. The existence of vestigial ectopic thymus not found in the dissected organs is considered the most likely hypothesis. Finally some considerations are made about a possible role of the rubeola virus on its etiology and the actual therapeutic possibilities.

Autopsy↗
PubMed1992

Acquired T cell specific deficiency other than acquired immunodeficiency syndrome (AIDS).

Evidence of an acquired T cell-specific deficiency distinct from acquired immunodeficiency syndrome (AIDS) in a 63-yr-old Japanese female is provided. Recently, this patients suffered from primary invasive pulmonary aspergillosis. Skin tests to purified protein derivative of tuberculin (PPD) and Aspergillus antigens were negative. Upon admission to our hospital, her lymphocytes were exclusively unresponsive to T cell mitogens (concanavalin A, phytohemagglutinin, and OKT 3). The level of cells defined by monoclonal antibodies (CD1, CD2, CD3, CD4, WT31, and CD5) was less than 3%. In contrast, no decrease in the number of red blood cells, platelets, neutrophils or B cells was apparent. Five years ago, the patient had a normal white blood cell and lymphocyte count. However, over the following 4 yr, she developed lymphopenia. With medication, her pulmonary disease recovered, while lymphopenia still continued. The levels of immunoglobulins, complements and enzyme activities (adenosine deaminase and purine nucleoside phosphorylase) were normal. Moreover, several tests for HIV (ELISA and Western bolt) were negative suggesting that the T cell-specific deficiency was not a congenital immunodeficiency or AIDS but rather a new type of acquired immunodeficiency.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Bone marrow transplantation in DiGeorge syndrome.

A Hispanic infant girl with DiGeorge syndrome underwent successful bone marrow transplantation (BMT) at age 28 1/2 weeks. She had typical facies, a cardiac defect, hypoparathyroidism, severe T and B cell immunodeficiency, and low levels of facteur thymique serique (FTS). In vitro incubation of the peripheral blood lymphocytes with thymosin alpha 1 showed no increase in the number of T cells on two occasions. A fetal thymus for transplantation was not available, and further review of past experience with thymic cells or factors revealed inconsistent and incomplete responses. Because of the patient's worsening clinical and immunologic status, BMT was performed, with her histocompatible brother as donor. The patient has had a good clinical and immunologic response to BMT, with evidence of T cell engraftment, improved B cell function, and increased levels of serum FTS. This experience indicates that minimal thymic influence is necessary for successful BMT and that patients with DiGeorge syndrome with significant T cell deficiency may benefit from this treatment.

Adult↗
PubMed1985

[Di George's syndrome complicated by graft versus host reaction].

On the occasion of a case of Di George syndrome, complicated by a post-transfusion lethal graft versus host reaction, the authors review its main clinical and biologic criteria. This case also shows that immune deficiencies should be diagnosed early in order to adopt preventive measures before transferring the patient in a specialized unit, where immunorestitution will be discussed.

DiGeorge Syndrome↗
PubMed1987

Regulation of the human IgE antibody response.

The frequent association of elevated serum IgE in patients with T cell immunodeficiencies suggest a role for T cells in the regulation of the human IgE antibody response. Unlike the situation with other isotypes the polyclonal B cells activators, pokeweed mitogen and Epstein Barr virus, do not routinely induce IgE synthesis in normal B cells. However, B cells from normal donors will synthesize immunoglobulins of all isotypes (including IgE) when cultured with T cell clones that recognize determinants expressed on the B cells (cognate stimulation). T cells with Fc receptors for IgE can be isolated from patients with hyper IgE syndrome and maintained as long term continuous T cell lines. These cells secrete IgE binding factors which enhance IgE synthesis but not IgG synthesis by preactivated IgE bearing B cells from allergic subjects but not resting B cells from normal donors. IgE binding factors isolated from sera of normal donors selectively suppress IgE synthesis. In contrast, IgE binding factors isolated from sera of patients with hyper IgE syndrome contain IgE potentiating activity as well as IgE suppressor activity. These results suggest that IgE synthesis in man is activated by T cells and isotype specific secretion of this immunoglobulin is modulated by IgE binding factors.

B-Lymphocytes↗