PLATELET AGGREGATION AND VWF DEFECTS IN HERMANSKY PUDLAK SYNDROME

Hermansky-Pudlak综合征（HPS）患者有轻微至中度的 血淋淋的历史我们已经检查了血小板功能，特别是 血小板聚集和释放反应，在这些患者中，发现 它们的致密颗粒含量不足。我们测量了血浆 血小板血管性血友病因子和其他血小板α颗粒 HPS患者血小板裂解物中的PF-4和β-TG等成分。 我们还在研究出血时间与临床 有血小板和血浆vWf活性和抗原的出血史 程度.在未来，我们计划检查HPS中的α-颗粒含量 患者，采用流式细胞术。

Patients with Hermansky-Pudlak Syndrome (HPS) have minor to moderate bleeding histories. We have examined platelet function, in particular platelet aggregation and release reaction, in these patients and found that they have deficient dense granule contents. We have measured plasma and platelet von Willebrand factor and other platelet alpha-granule constituents such as PF-4 and beta-TG in platelet lysates of HPS patients. We are also in the process of correlating bleeding times and clinical bleeding histories with platelet and plasma vWf activity and antigen levels. In the future, we plan to examine alpha-granule contents in HPS patients, employing flow cytometry.