[Association of Sweet syndrome and salmonellosis].
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Biomedical subjects
Publications and source records attributed to M Karmochkine.
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A previously healthy 34-year-old woman, was diagnosed as having systemic lupus erythematosus (SLE), with membranous glomerulopathy which improved rapidly. Neither lupus anticoagulant nor anticardiolipin antibodies were detected in her plasma. After three months of total remission, she developed a severe pulmonary thromboembolism for which no specific biological cause was found. Her plasma was analysed for different antiphospholipid antibodies: lupus anticoagulant and anticardiolipin antibodies were again negative. Using an ELISA prepared with either five different anionic phospholipids or zwitterionic phosphatidylethanolamine, solely an anti-phosphatidylethanolamine IgG was discovered in her plasma. In lupus patients, the presence of antiphospholipid antibodies is now well recognized as a high risk factor for repeated thrombosis and/or recurrent abortions. This case suggests that the presence of antiphosphatidylethanolamine antibody should be investigated in cases of unexplained thrombosis in SLE, where the usual clinical and biological investigations have failed to shed light.
OBJECTIVES: To investigate the phenotype of lymphocytes and dendritic cell precursors in the peripheral blood of adult patients with histiocytosis X. METHODS: Data were obtained on patients with histiocytosis X treated in La Pitié-Salpetrière Hospital. Peripheral blood mononuclear cells from 10 patients (4 with unifocal and 6 with disseminated disease) were studied by flow cytometry. A method was set up to detect circulating Langerhans cells, dendritic cells and their precursors. RESULTS: An abnormal repartition of "memory" T lymphocyte subsets was observed, with a significant decrease of CD4CD45RO and CD8CD45RO and a reciprocal increase of CD4CD45RA "naive" cells, while CD4+ cells displaying the accessory molecule CD28 were decreased in some patients. These abnormalities disappeared in vitro after triggering of the CD3 and CD28 molecules in the absence of antigen presenting cells, hence demonstrating that there was no constitutive defect in the capacity of CD4+ and CD8+ T cells to convert from the CD45RO- to the CD45RO+ isoform. Langerhans cells were undetectable in the peripheral blood, and dendritic cells and their precursors were present in normal proportions (0.5 +/- 0.2% and 2.8 +/- 1.2%, respectively), but the latter were more numerous (4% and 6% of the PBMC) in the two patients with the more severe form of the disease. CONCLUSIONS: We found in these patients some T lymphocyte phenotype abnormalities which suggest alterations in antigen-driven activation processes. The number of dendritic precursor cells was not consistently elevated in the peripheral blood from histiocytosis X patients.