[Angioimmunoblastic lymphadenopathy (AIL) rich in epithelioid cells presenting a Gougerot-Sjögren syndrome. Nosological relationship between Sjörgen "pseudolymphomas" and AIL (a case with a fatal course)].
The authors report the case of a 62-year-old woman in whom, 18 months following the development of bilateral parotid hypertrophy followed by a dry syndrome, there was the onset of an angioimmunoblastic lymphadenopathy (AIL) which consisted of the association of a multiple lymphadenopathy with a characteristic histological appearance, although poor in plasmocytes, and a febrile syndrome, but in the absence of a complete syndrom from a laboratory standpoint. This fact, incombination with the richness of the nodes in epitheloid cells, is such that this case resembles the type II "dysimmune and pseudo-lymphomatous lymphadenopathies" (DPLL) of Delsol et al. Although the term AIL has never been used before in the title of previous publications of pseudo-lymphomas occuring during Sjögren's syndrome, it would seem possible, as has already been suggested by Diebold et al. (3) with regard to several cases, that certain of these pseudo-lymphomas are true AIL. The rapidly fatal course with visceral spread shown at autopsy and above all the presence of exclusively immunoblastic plaques in several mode areas led, on the basis of the criteria of Nathwani et al. (22), to acceptance of the coexistence of an immunoblastic sarcoma.
D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000175:diagnosis / Q000276:immunology / Q000473:pathology*; D007136:Immunoglobulins / Q000032:analysis; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D012859:Sjogren's Syndrome / Q000175:diagnosis
D007136:Immunoglobulins
Lymphadénopathie angio-immunoblastique (L.A.I.) riche en cellules épithélioïdes révélée par un syndrome de Gougerot-Sjögren. Rapports nosologiques des "pseudo-lymphomes" du Sjögren avec la L.A.I. (à propos d'un cas d'évolution mortelle).