[Long-term remission after i.v. immunoglobulin therapy in acquired antihemophilic factor hemophilia with systemic lupus erythematosus].
[Long-term remission after i.v. immunoglobulin therapy in acquired antihemophilic factor hemophilia with systemic lupus erythematosus].
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[静脉注射后长期缓解
DOI：
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发表时间：
1990
影响因子：
1
通讯作者：
B. Manger
中科院分区：
文献类型：
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作者：
K. Pirner;W. Rösler;J. Kalden;B. Manger
1例27岁女性，严重系统性红斑狼疮合并肾脏受累，确诊后5年出现大面积皮肤出血。常规凝血试验证实活化的部分凝血活酶时间延长至77 S。这归因于凝血因子VIII活性显着降低至不到3%。确定了一种对因子VIII的抑制活性为1.4贝塞斯达单位的抑制剂。免疫抑制治疗(激素、硫唑嘌呤、环磷酰胺、环孢素)对出血无影响。后来在病程中出现危及生命的阴道出血，同时出现狼疮活动的发作。期间采用S静脉滴注治疗7例。开始使用免疫球蛋白(120g，5d内)。这导致出血立即止住。凝血因子VIII活性在7天内从3%上升到480%，双链DNA抗体从122U/ml下降到19.7U/ml。9个月后，在环磷酰胺和类固醇的免疫抑制治疗下，第八因子活性仍在正常范围内，没有发生出血事件。这证实了大剂量免疫球蛋白治疗血友病的有效性，由于获得性因子抗体，在重症SLE患者中也是如此。
A 27-year-old female with severe systemic lupus erythematosus with renal involvement developed extensive cutaneous hemorrhages 5 years after diagnosis. Routine coagulation tests confirmed a prolongation of activated partial thromboplastin time to 77 s. This was attributed to a marked reduction of factor VIII activity to less than 3%. An inhibitor with an activity of 1.4 Bethesda units against factor VIII was determined. Immunosuppressive therapy (steroids, azathioprin, cyclophosphamide, cyclosporine) had no influence on the hemorrhages. Later in the course of disease a life-threatening vaginal hemorrhage occurred in parallel with a flare-up of lupus activity. During that period a therapy of 7 S i.v. immunoglobulins (120 g within 5 days) was started. This led to an instant cessation of the bleeding. Factor-VIII activity rose from 3% ot 480% within 7 days and the ds-DNA-antibodies fell from 122 U/ml to 19.7 U/ml. Nine months later, under immunosuppressive therapy with cyclophosphamide and steroids, factor-VIII activity is still within the normal range and no bleeding episodes have occurred. This confirms the effectively of high-dose immunoglobulin therapy for hemophilia, due to acquired factor VIII antibodies, also in patients with severe SLE.