Ittyerah R, Alkjaersig N, Fletcher A, Chaplin H
J Lab Clin Med. 1976 Oct;88(4):546-54.
Plasma factor XIII was assayed serially for a 1 1/2 to 2 year period in four patients with sickle-cell disease (SSD), during which several episodes of SSD crisis were observed. During crisis-free periods, plasma factor XIII concentration was lower than in normal subjects. With crisis onset, there was usually a concomitant fall in platelets and in plasma factor XIII soon after followed by a rise in fibrinogen concentration and platelet count. Following crisis resolution there was an increase in plasma factor XIII concentration peaking 3 weeks after crisis onset and occurring at the time of subsidence of thrombocytosis. It is suggested that the observed changes in plasma factor XIII concentration in SSD patients support the hypothesis that in the early stage of SSD crisis, fibrin deposition and/or thrombosis significantly enhance the role of sickled erythrocytes in producing vascular occlusion and/or organ infarction.
在4例镰状细胞病（SSD）患者中，连续1.5至2年对血浆因子XIII进行检测，在此期间观察到几次SSD危象发作。在无危象期，血浆因子XIII浓度低于正常受试者。随着危象发作，血小板和血浆因子XIII通常会随之下降，随后纤维蛋白原浓度和血小板计数会升高。危象缓解后，血浆因子XIII浓度会升高，在危象发作后3周达到峰值，且发生在血小板增多症消退之时。有人提出，SSD患者血浆因子XIII浓度的上述变化支持这样一种假说，即在SSD危象的早期阶段，纤维蛋白沉积和/或血栓形成会显著增强镰状红细胞在导致血管闭塞和/或器官梗死方面的作用。
