Finazzi G, Budde U, Michiels J J
Hematology Division, Ospedali Riuniti, Bergamo, Italy.
Leuk Lymphoma. 1996 Sep;22 Suppl 1:71-8. doi: 10.3109/10428199609074363.
Bleeding time (BT) and platelet function tests have been widely used in patients with essential thrombocythemia (ET), with the aim to support diagnosis and to identify laboratory predictors of haemorrhagic and thrombotic complications. BT is significantly prolonged in 7-19% of ET patients and several functional abnormalities have been observed in platelet structure, biochemistry and survival. However, the attempt to relate these in vivo and in vitro platelet dysfunctions with diagnosis or clinical sequelae has been generally disappointing. Therefore, BT and platelet function tests are currently not recommended in the initial evaluation or during the follow-up of patients with ET, unless in the setting of a clinical or biological study. A noteworthy exception is represented by a subset of patients characterized by very high platelet count (> 1500 x 10(9)/L) and bleeding symptoms, who can have an acquired von Willebrand disease. In these cases, prolonged BT and abnormal multimeric pattern of von Willebrand factor are useful for diagnosing and monitoring this acquired hemorrhagic disease. BT and platelet function tests should be included in the baseline evaluation of ET patients enrolled in prospective clinical trials aiming assess their predictive role on clinical end-points.
出血时间（BT）和血小板功能检测已广泛应用于原发性血小板增多症（ET）患者，旨在辅助诊断并确定出血和血栓形成并发症的实验室预测指标。7%至19%的ET患者出血时间显著延长，并且在血小板结构、生物化学和存活方面观察到了一些功能异常。然而，将这些体内和体外血小板功能障碍与诊断或临床后果联系起来的尝试总体上令人失望。因此，目前不建议在ET患者的初始评估或随访期间进行BT和血小板功能检测，除非是在临床或生物学研究的背景下。一个值得注意的例外是一部分血小板计数非常高（>1500×10⁹/L）且有出血症状的患者，他们可能患有获得性血管性血友病。在这些情况下，延长的BT和血管性血友病因子的异常多聚体模式有助于诊断和监测这种获得性出血性疾病。对于参与旨在评估其对临床终点预测作用的前瞻性临床试验的ET患者，BT和血小板功能检测应纳入基线评估。
