[Immunologic study of familial lymphohistiocytosis. Eight new case reports (author's transl)].
We report 8 cases of familial lymphohistiocytosis collected in 6 families. Several data argue for an hyperactivation of the reticuloendothelial system (RES). An abnormal visualization of all organs was observed in a scintigraphic study after 99technetium labelled red blood cells injection. Blood monocytes contained very low peroxidase activity as detected by cytoenzymology and secreted large quantities of prostaglandin E2 (PGE2). Adherent cells isolated from blood exercised a strong suppressor effect on the proliferation of normal lymphocytes induced by phytohemaglutinin. This effect was reduced by indomethacin and therefore appears PGE2-dependent. One patient's serum exerced an inhibitory activity on antigen-induced proliferation of normal lymphocytes and on mixed leucocyte reaction. In contrast, cellular and humoral functions were not deeply impaired. The hyperactivation of the RES remains unexplained and not related to a graft versus host reaction, which could be excluded in 3 of our patients. Therapeutic attempts were not efficient, all patients dying despite steroid, vincaleucoblastin and indomethacin therapy.
D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008055:Lipids / Q000097:blood; D008206:Lymphatic Diseases / Q000097:blood / Q000235:genetics* / Q000276:immunology; D008297:Male; D009000:Monocytes / Q000502:physiology
D008055:Lipids
Etude immunologique de la lymphohistiocytose familiale. A propos de huit nouvelles observations.