Résumé
Objective To summarize the manifestation and treatment of acquired hemophilia A in pa- tients with systemic lupus erythematosus(SLE).Method A case was investigated retrospectively and the lit- erature was reviewed.Results A 25-year-old woman with a 5 year history of SLE was admitted to hospital due to abdominal pain.She was diagnosed with acquired factorⅧinhibitor deficiency based on a prolonged activated partial-thromboplastin time(APTT,135.3 s),reduced factorⅧactivity(0.9%)and factorⅧin- hibitor(26.1 BU/ml).Sonography and magnetic nuclear resonance of the abdomen confirmed the presence of a retro-uterine hematoma.The patient was initially treated with intravenous pulse and oral corticosteroids,factorⅧplasma concentrated and intravenous immunoglobulin.Clinical and biological improvement was promptly obtained.Conclusions Attention should be paid to the association between SLE and acquired hemophilia A. Combination therapy may be recommended as initial therapy for the management of acquired hemophilia A in patients with SLE.But no standardized treatment can be recommended at present.
Résumé
Objective To investigate the expression and clinical significance of peripheral blood CD4~+, CD25~+ and CD4~+CD25~+ T subpopulations in patients with systemic lupus erythematosis.Methods The per- centage and fluorescence intensities of peripheral blood CD4~+,CD25~+ and CD4~+CD25~+ subpopulations from 34 SLE and 18 normal controls were measured with flow cytometry assay,then the correlation with clincal data was analyzed.The CD25~+ cells were defined as the CD25~(high) cells if their fluorescence intensity was higher than 10. Results The percentage of CD4~+CD25~+,CD4~+CD25~(high) T lymphocytes in active SLE patients[(4.80?1.21)% and (0.25?0.10)%]was lower than that in normal controls[(8.92?3.21)% and(0.44?0.22)% and non-active SLE patients(11.28?2.09)% and(0.59?0.34)%](P<0.05).However,as for the CD25~+ cells in the CD4~+ T cells,there was no difference between SLE patients and normal control group.Peripheral blood CD4~+CD25~+,CD4~+CD25~(high) cells in SLE were reversely correlated with SLEDAI(r=-0.74,P=0.004 and r=-0.614,P=0.026),but not with others such as complements,ANA titers etc.Peripheral blood CD4~+ and CD25~+ lymphocytes in active SLE pa- tients were also lower than those in normal controls[(23?7)vs(34?7)and(7.4?1.8)vs(13.9?3.4),P<0.05]. CD25 fluorescence intensities were higher in the SLE patients those in the normal controls,but CD4 fluores- cence intensities were not.Conclusion CD4~+CD25~+ may play a role in the pathogenesis of SLE.