Jelenska M, Kopeć M, Breddin K
Haemostasis. 1985;15(3):169-75. doi: 10.1159/000215140.
Fibrin clot retraction (FCR) and collagen gel retraction (CGR) were studied in patients with inherited platelet defects, i.e. in Glanzmann's thrombasthenia, Hermansky-Pudlak syndrome, May-Hegglin anomaly, giant platelet syndrome, as well as in patients with von Willebrand disease and factor XIII deficiency. FCR was abnormal only in thrombasthenia, while CGR was found to be reduced in 2 patients with Hermansky-Pudlak syndrome and in 4 out of 5 cases with von Willebrand disease. Both FCR and CGR were normal in May-Hegglin anomaly, giant platelet syndrome and severe factor XIII deficiency. In none of the examined bleeding disorders was a concomitant FCR and CGR reduction detected. Natural polyamines and anti-fibronectin antibodies did not affect platelet potency in FCR or CGR. Peroxidation of platelet membrane components by sodium periodate abolished the platelet-induced FCR and CGR. This effect was reversed by subsequent reduction by sodium borohydride.
对患有遗传性血小板缺陷的患者，即患有Glanzmann血小板无力症、Hermansky-Pudlak综合征、May-Hegglin异常、巨大血小板综合征的患者，以及患有血管性血友病和因子XIII缺乏症的患者，研究了纤维蛋白凝块回缩（FCR）和胶原凝胶回缩（CGR）。FCR仅在血小板无力症中异常，而在2例Hermansky-Pudlak综合征患者和5例血管性血友病患者中的4例中发现CGR降低。May-Hegglin异常、巨大血小板综合征和严重因子XIII缺乏症患者的FCR和CGR均正常。在所检查的出血性疾病中，均未检测到FCR和CGR同时降低。天然多胺和抗纤连蛋白抗体不影响血小板在FCR或CGR中的效能。高碘酸钠对血小板膜成分的过氧化作用消除了血小板诱导的FCR和CGR。随后用硼氢化钠还原可逆转这种作用。
