[Job syndrome (hyper-IgE) and hypo-IgA. A rare association of immunodeficiencies].
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.
Mazzone
A
A
Dipartimento di Medicina Interna e Terapia Medica, Università IRCCS, Policlinico San Matteo, Pavia.
Girola
S
S
Fossati
G
G
Mazzucchelli
I
I
Ricevuti
G
G
D000328:Adult; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D005260:Female; D006801:Humans; D017098:IgA Deficiency / Q000175:diagnosis / Q000276:immunology*; D007111:Immunity, Cellular; D007070:Immunoglobulin A / Q000097:blood; D007073:Immunoglobulin E / Q000097:blood; D007589:Job Syndrome / Q000175:diagnosis / Q000276:immunology*
D007070:Immunoglobulin A; D007073:Immunoglobulin E
Sindrome di Giobbe (Iper-IgE) ed ipo-IgA. Rara associazione di immunodeficienze.