Triadou P, Fonty E, Ambrosio A S, Cottat M C, Girot R, Cornu P
Laboratoire d'Hématologie, Hôpital Necker-Enfants Malades, Paris, France.
Nouv Rev Fr Hematol (1978). 1990;32(2):137-42.
Platelet function was investigated in 37 patients with sickle cell disease during steady state. The measurement of platelet aggregation in whole blood, demonstrating interaction of sickle cells and platelets, showed increased activity in patients compared to controls. In contrast, by classical platelet aggregation in platelet-rich plasma (PRP) we observed decreased platelet aggregation in sickle cell patients. Aggregation of washed platelets appeared identical in patients and controls beta thromboglobulin (beta TG) and platelet factor 4 (PF4) as well as fibrinopeptide A (FPA) plasma levels were increased in patients with sickle cell disease. These results suggest that in sickle cell patients there is in vivo platelet stimulation, which may therefore appear "exhausted" in patients plasma during in vitro studies, and also a possible role of coagulation in the pathophysiology of sickle cell disease as supported by high levels of FPA.
在37例镰状细胞病稳态期患者中对血小板功能进行了研究。全血中血小板聚集的测量显示了镰状细胞与血小板的相互作用，结果表明与对照组相比，患者的血小板聚集活性增加。相比之下，通过富含血小板血浆（PRP）中的经典血小板聚集试验，我们观察到镰状细胞病患者的血小板聚集减少。洗涤血小板的聚集在患者和对照组中表现相同。镰状细胞病患者的β-血小板球蛋白（β-TG）、血小板因子4（PF4）以及纤维蛋白肽A（FPA）血浆水平升高。这些结果表明，镰状细胞病患者体内存在血小板刺激，因此在体外研究中患者血浆中的血小板可能显得“耗竭”，同时高水平的FPA也支持凝血在镰状细胞病病理生理学中可能发挥的作用。
