Acquired von Willebrand Syndrome in a Male with Systemic Lupus Erythematosus Presented with Mucocutaneous Bleeding and Treated with rFVIIa

Background: Systemic lupus erythematosis (SLE) is a disorder with multiple organ involvement. Haematological abnormalities have been addressed in it, but acquired von Willebrand syndrome is a rarer phenomenon in current disease. The Case: We report acquired von Willebrand syndrome and SLE in a man with brown rash on face, gingival bleeding, easy bruising and epistaxis and laboratory finding of decreased complement, high level of anti-nuclear antibody and anti-DNA. These findings confirmed the diagnosis of SLE. He underwent kidney biopsy and experienced severe pain at the site of biopsy, but the ultra-sonography evaluation showed small sub capsular haematoma at the site of biopsy. During the next 48 hours, gradually APTT prolongation was continued and haematocrit dropped. In spite of FFP infusion and taking tranexamic acid every eight hours, there wasn’t any improvement in haemostatic condition. He received Methylprednisolone and Cyclophosphamid pulses. The patient underwent surgery to roll out vascular complication, but there wasn’t any vascular problem. On the third day, recombinant activated factor VII was infused every two hours until oozing was stopped.

Effective prophylaxis with rFVIIa in young haemophiliacs with inhibitors using a schedule similar to FVIII prophylaxis in non-inhibitor patients

Objective: To assess the role of early prophylaxis with recombinant activated factor VII (rFVIIa) in young haemophiliacs with inhibitors and to determine whether it can reduce bleeding episodes and prevent joint damage. Patients and Methods: Ten severe haemophiliacs, less of three years old, with up to four joint bleeds and inhibitors to FVIII who started early prophylaxis with rFVIIa, were included. A number of haemorrhages/year/patient and haemarthroses/ year/patient were compared before the start of prophylaxis, which include both the time before (initial period) and after inhibitor diagnosis (inhibitor period), with those during prophylaxis (prophylaxis period). Results: The mean time of inhibitor diagnosis was 15.6 months (range: 2.3-34.1). The mean time between inhibitor diagnosis and the start of prophylaxis was 7.1 months (range: 0-23.2), shorter than the time of rFVIIa prophylaxis (mean: 10.3 months;range: 4.1-32.0). Bleeding episodes for the three time periods were 45, 36 and 17, respectively, or 0.29 and 0.51 haemorrhages/year/patient in the two periods prior to prophylaxis compared to 0.17 during prophylaxis. Total haemarthroses for the three-time periods were 7, 5 and 5, respectively. The haemarthroses/year/patient in the pre-prophylaxis period were 0.032 and 0.070, compared to0.049 inthe prophylaxis period. rFVIIa schedules were either 90 μg/kg three times weekly or 90 μg/kg daily. Conclusions: Early prophylaxis with rFVIIa may be efficacious in young haemophiliacs with inhibitors, reducing joint bleeds. After the risky period of inhibitor presence, they are able to continue rFVIII prophylaxis with success assured to prevent arthropathy.

重组活化凝血因子Ⅶ在脑出血早期应用疗效的系统评价

目的：评价重组活化凝血因子Ⅶ在脑出血早期治疗中的疗效及安全性。方法计算机检索Pubmed、Medline、Web of knowledge、万方、CHKI等中外文献数据库，收集有关重组活化凝血因子Ⅶ在脑出血早期应用的随机对照试验。对纳入的文献按Cochrance系统评价方法进行质量评估，使用Revman5软件对文献数据进行整理分析。结果共纳入7篇研究，共1493例患者。Meta分析发现，剂量为40μg/kg的rFⅦa治疗组与对照组比较，脑出血患者的病死率明显降低（P〈0.05），血肿增加量差异无统计学意义，神经功能恢复明显改善（P〈0.05），不良事件发生差异无统计学意义。剂量为80μg/kg的rFⅦa治疗组与对照组，脑出血患者血肿增加量明显减少（P〈0.05），病死率、神经功能恢复以及不良事件发生差异均无统计学意义。结论脑出血早期治疗中应用剂量为40μg/kg的rFⅦa可明显降低患者病死率，改善患者预后。剂量为80μg/kg的rFⅦa治疗可减少血肿扩大发生，同时rFⅦa具有良好的安全性。

Vaginal Delivery in a Primipara with Glanzmann Thrombasthenia

To editor:Glanzmann thrombasthenia(GT)is a rare autosomal recessive bleeding disorder that is characterized by a quantitative and/or qualitative defect in the platelet integrinαIIbβ3(previously known as glycoprotein(GP)IIb/IIIa),the major platelet receptor of fibrinogen.DefectiveαIIbβ3 can result in the absence of platelet aggregation.Pregnancy and delivery in women with GT can present specific challenges as there is a significant risk of both maternal and fetal bleeding.

脑出血后早期血肿增大的研究进度

脑出血后早期血肿增大可独立预测其不良预后。本文从早期血肿增大的发生率、对预后的影响、血肿增大相关临床因素、病理生理机制、分子信号及rFVIIa治疗等方面进行综合阐述。