Literature DB >> 8725083

[Job syndrome (hyper-IgE) and hypo-IgA. A rare association of immunodeficiencies].

A Mazzone1, S Girola, G Fossati, I Mazzucchelli, G Ricevuti.   

Abstract

Job' syndrome and IgA immunodeficiency are a rare dysfunction of the immune system. In this work, we reported a case of a young woman who had recurrent episodes of bacterial infections in the urinary tract and genital, generalized erythematous eczematous patches and stomatitis of oral mucosa and fever. During the hospitalization, laboratory data showed high immunoglobulin IgE and low IgA levels. The T-lymphocyte presented a reduction of CD8+ cells. Tests of granulocyte function have showed a global deficit in the in vitro and in vivo chemotaxis. The correlation between these two clinic conditions is not completely clarified but it is possible to hypothesize that CD8+ lymphocytes produce an inhibition factor of chemotaxis. Job' syndrome is characterized by a selective reduction of CD8+ cells subpopulation which have an immunoregulatory function on the production of IgE by plasmacells. In the ipoIgA, an intrinsic inability of B-IgA cells to proliferate and to differentiate produce a defect in the IgA production. In these two clinic disorders there is an effective dysfunction of immune system. It is possible to hypothesize that an effective defect of CD8+ cells and an immaturity of B-cells may coexist in our patient. That justifies an abnormal production of Ig and a defect in granulocyte chemotaxis.

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Year:  1996        PMID: 8725083

Source DB:  PubMed          Journal:  Recenti Prog Med        ISSN: 0034-1193


  1 in total

1.  Lessons learned from phagocytic function studies in a large cohort of patients with recurrent infections.

Authors:  Baruch Wolach; Ronit Gavrieli; Dirk Roos; Sivan Berger-Achituv
Journal:  J Clin Immunol       Date:  2011-12-30       Impact factor: 8.317

  1 in total

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