Al-Mondhiry H
Department of Medicine, Pennsylvania State University, College of Medicine, Hershey.
Thromb Haemost. 1987 Jun 3;57(3):294-7.
Platelet function abnormalities have been described in some patients with hemophilia. This study reports measurements of plasma beta-thromboglobulin (BTG) and platelet factor 4 (PF4), sensitive and specific markers of platelet alpha-granule release in 72 hemophiliacs. Patients were studied on multiple occasions depending on their clinical condition and the treatment given. Stable patients without recent bleeding showed significantly elevated BTG levels: 29.72 +/- 12.77 ng/ml, control 23.22 +/- 8.22, p = 0.006. This appears to be independent of the presence of liver disease, human immune deficiency virus infection (HIV) and the acquired immune deficiency syndrome (AIDS). The increased BTG level seems to correlate with the severity of the disease as reflected by the frequency of bleeding and the quantity of factor concentrate used but not with plasma factor VIII or IX procoagulant activity. Patients who have experienced recent bleeding showed a marked rise in BTG level (77.26 +/- 51.37 ng/ml, p = 0.0002), indicating enhanced in vivo platelet activation. These observations suggest that enhanced platelet release in hemophilia most likely reflects sustained activation of hemostasis secondary to frequent bleeding characteristic of the severe form of the disease.
一些血友病患者存在血小板功能异常。本研究报告了72例血友病患者血浆β-血小板球蛋白（BTG）和血小板因子4（PF4）的测量结果，这两种物质是血小板α-颗粒释放的敏感且特异的标志物。根据患者的临床状况和所接受的治疗，对他们进行了多次研究。近期无出血的稳定患者显示BTG水平显著升高：29.72±12.77 ng/ml，对照组为23.22±8.22，p = 0.006。这似乎与肝病、人类免疫缺陷病毒感染（HIV）和获得性免疫缺陷综合征（AIDS）的存在无关。BTG水平的升高似乎与疾病的严重程度相关，具体表现为出血频率和所使用的凝血因子浓缩物的量，但与血浆因子VIII或IX促凝活性无关。近期有出血经历的患者BTG水平显著升高（77.26±51.37 ng/ml，p = 0.0002），表明体内血小板激活增强。这些观察结果表明，血友病患者血小板释放增强很可能反映了由于该疾病严重形式的频繁出血所导致的止血过程的持续激活。
