系统性红斑狼疮患者继发获得性血管性血友病1例  

Secondary Acquired von Willebrand after Systemic Lupus Erythematosus

作　　者：王冰[1] 刘雪艳[1] 郑秀芬[1] 钟彩梅[2] 曾抗[1] 孙乐栋[1] 

机构地区：[1]南方医科大学南方医院皮肤科,广东广州510515 [2]南方医科大学珠江医院皮肤科,广东广州510282

出　　处：《中国皮肤性病学杂志》2011年第12期979-980,共2页The Chinese Journal of Dermatovenereology

摘　　要：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%。A 25-year-old female patient with systemic lupus erythematosus 6 years, has abdominal pain, nausea, vomiting and melena half a month ago. Rectal examination showed a cystic mass right after the uterus up to 8cm ×7cm ×6cm, with tenderness and poor activity. About 2mL dark red blood was found in it when undergoing B transabdominal pelvic ultrasound-guided puncture. With administered prednisone and leflunomide as 50, 20mg/day respectively, and 40mg Omeprazole Sodium injection necessarily, her symptoms improved. But soon there were scattered petechia and ecchymosis all over the body ranging from the fingertips to bean. Blood tests showed PLT 289 ×10^9/L, plasma procoagulant activity of factor Ⅷ 0.9%, yon Willebrand factor antigen 2.3% and ristocetin-induced platelet aggregation test 3.8%. So, the patient was diagnosed as systemic lupus erythematosus with secondary acquired von Willebrand disease. As a result of adding immunoglobulin （400mg/kg ×5days） , fresh plasma and plasma factor Ⅷ to the treatment, she alleviated. During the 1-year-follow up, the systemic lupus erythematosus in the patient was stable with a gradual reduction of prednisone to 20mg / day, besides, there was no bleeding symbol, with plasma procoagulant activity of factor Ⅷ, yon Willebrand factor antigen ievel and ristocetin-induced platelet aggregation test results were 79.6%, 92.1% and 112.6% ,respectively.

关 键 词：红斑狼疮 系统性 获得性血管性血友病 

分 类 号：R593.24[医药卫生—内科学]