We describe two previously healthy children who had multiple ecchymoses several days after acute infection. In both cases,the prothrombin time (PT) and the activated partial thromboplastin time (APTT) were prolonged. ...We describe two previously healthy children who had multiple ecchymoses several days after acute infection. In both cases,the prothrombin time (PT) and the activated partial thromboplastin time (APTT) were prolonged. Further examinations revealed the presence of lupus anticoagulant (LA), phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT), and low serum complement. In both cases, we confirmed the presence of a serum immune complex. The patients’ symptoms improved spontaneously within 1 week, and all laboratory data normalized within several months. We also describe another asymptomatic case positive for LA and aPS/PT presumably associated with cytomegalovirus infection. The prevalence of transient antiphospholipid antibodies associated with viral infections in children must be much higher than we expected.We have to take it into consideration when we see abnormalcoagulation results, but the occurrence of significant bleeding symptoms is rare.展开更多
Objective:To describe the safety and efficacy of rituximab inthe treatment of childhood-onset systemic lupus erythematosus(SLE). Study design:We conducted a French multicenter retrospective study of childhood-onset SL...Objective:To describe the safety and efficacy of rituximab inthe treatment of childhood-onset systemic lupus erythematosus(SLE). Study design:We conducted a French multicenter retrospective study of childhood-onset SLE treated with rituximab.Results:Eleven girls with severe SLE,including 8 girls with class IV or V lupus nephritis,2 girls with severe autoimmunecytopenia,and 1 girl with antiprothrombin antibody with severe hemorrhage,were treated with rituximab. The meanage at onset of rituximab treatment was 13.9 years. Patients received 2 to 12 intravenous infusions of rituximab (350-450 mg/m2/infusion),with corticosteroids. Six patients also received different standard immunosuppressive agents,including Cyclophosphamide (2 patients). Remission was achieved in 6 of 8 patients with lupus nephritis and in the 2 patients with autoimmunecytopenia. Steroid therapy was tapered in 5 patients who responded to treatment,and low-dose prednisone treatment was maintained in 1 patient. The mean follow-up period was 13.2 months (range,6-26 months),and remission lasted in all who patients who responded to treatment,except 1 patient who was successfully retreated with a second course of rituximab.Anti-double-stranded DNA antibody levels decreased in 6 of11 patients,and anticardiolipin antibody levels decreased in 3 of 4 patients. Severe adverse events developed in 5 patients.Effective depletion of peripheral blood B cells was observed in 7 of 8 patients who were examined,and this paralleled the remission. Conclusion:Rituximab may be an effective cotherapy;however,further investigations are required because severe adverse events occurred in 45%of the patients in this study.展开更多
文摘We describe two previously healthy children who had multiple ecchymoses several days after acute infection. In both cases,the prothrombin time (PT) and the activated partial thromboplastin time (APTT) were prolonged. Further examinations revealed the presence of lupus anticoagulant (LA), phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT), and low serum complement. In both cases, we confirmed the presence of a serum immune complex. The patients’ symptoms improved spontaneously within 1 week, and all laboratory data normalized within several months. We also describe another asymptomatic case positive for LA and aPS/PT presumably associated with cytomegalovirus infection. The prevalence of transient antiphospholipid antibodies associated with viral infections in children must be much higher than we expected.We have to take it into consideration when we see abnormalcoagulation results, but the occurrence of significant bleeding symptoms is rare.
文摘Objective:To describe the safety and efficacy of rituximab inthe treatment of childhood-onset systemic lupus erythematosus(SLE). Study design:We conducted a French multicenter retrospective study of childhood-onset SLE treated with rituximab.Results:Eleven girls with severe SLE,including 8 girls with class IV or V lupus nephritis,2 girls with severe autoimmunecytopenia,and 1 girl with antiprothrombin antibody with severe hemorrhage,were treated with rituximab. The meanage at onset of rituximab treatment was 13.9 years. Patients received 2 to 12 intravenous infusions of rituximab (350-450 mg/m2/infusion),with corticosteroids. Six patients also received different standard immunosuppressive agents,including Cyclophosphamide (2 patients). Remission was achieved in 6 of 8 patients with lupus nephritis and in the 2 patients with autoimmunecytopenia. Steroid therapy was tapered in 5 patients who responded to treatment,and low-dose prednisone treatment was maintained in 1 patient. The mean follow-up period was 13.2 months (range,6-26 months),and remission lasted in all who patients who responded to treatment,except 1 patient who was successfully retreated with a second course of rituximab.Anti-double-stranded DNA antibody levels decreased in 6 of11 patients,and anticardiolipin antibody levels decreased in 3 of 4 patients. Severe adverse events developed in 5 patients.Effective depletion of peripheral blood B cells was observed in 7 of 8 patients who were examined,and this paralleled the remission. Conclusion:Rituximab may be an effective cotherapy;however,further investigations are required because severe adverse events occurred in 45%of the patients in this study.
