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患者女,25岁。诊断为系统性红斑狼疮6年,下腹痛、恶心、呕吐伴有黑便半月。入院时肛门指检示子宫后可触及8cm×7cm×6cm大小的囊性包块。B超引导下经腹盆腔穿刺,抽出暗红色不凝血约2mL。予泼尼松50mg/d、来氟米特20mg/d及注射用奥美拉唑钠等治疗,症状好转。但不久全身出现散在的指尖到蚕豆大小不等瘀点、瘀斑。查血常规示血小板289×109/L,血浆因子Ⅷ促凝活性为0.9%,von Willebrand因子抗原水平为2.3%,瑞斯托霉素诱导的血小板凝集试验结果为3.8%。诊断为系统性红斑狼疮继发获得性血管性血友病。加用人免疫球蛋白冲击治疗(400mg/kg×5d),补充新鲜血浆及血浆因子Ⅷ等治疗后,症状缓解。随访1年,患者系统性红斑狼疮病情稳定,泼尼松逐渐减量至20mg/d,未出现出血征象,复查血浆因子Ⅷ促凝活性为79.6%,von Willebrand因子抗原水平为92.1%,瑞斯托霉素诱导的血小板凝集试验结果为112.6%。
Female patient, 25 years old. Diagnosis of systemic lupus erythematosus for 6 years, lower abdominal pain, nausea, vomiting accompanied by melena half months. On admission, the anus refers to the uterus after detection can reach the size of 8cm × 7cm × 6cm cystic mass. B-guided abdominal pelvic puncture, dark red out of about 2mL. Prednisone 50mg / d, leflunomide 20mg / d and omeprazole sodium for injection and other treatment, the symptoms improved. But soon scattered body fingertips ranging from broad bean pests, ecchymosis. Blood tests routinely showed a platelet count of 289 × 109 / L, plasma factor Ⅷ procoagulant activity of 0.9%, von Willebrand factor antigen level of 2.3%, and ristocetin-induced platelet aggregation test of 3.8%. Diagnosis of systemic acquired lupus erythematous secondary acquired von Willebrand disease. Plus human immunoglobulin impact therapy (400mg / kg × 5d), add fresh plasma and plasma factor Ⅷ treatment, the symptoms relieved. Follow-up of 1 year, patients with systemic lupus erythematosus in stable condition, prednisone tapering to 20mg / d, no signs of bleeding, review of plasma factor VIII procoagulant activity was 79.6%, von Willebrand factor antigen level was 92.1% The tropomycin-induced platelet aggregation test result was 112.6%.