Rondeau E, Sraer J, Bens M, Doleris L M, Lacave R, Sraer J D
Eur J Clin Invest. 1987 Feb;17(1):53-7. doi: 10.1111/j.1365-2362.1987.tb01225.x.
Periodic systemic capillary leak syndrome (Clarkson disease) is characterized by unexplained attacks of a marked increase in capillary permeability. As leukotrienes, derived from arachidonic acid via the 5-lipoxygenase pathway, enhance capillary permeability, we studied arachidonate metabolism in leucocytes of a patient with capillary leak syndrome. Leucocyte-platelet suspensions, prepared from blood collected from the patient during asymptomatic periods (n = 11) produced greater amounts of 5-hydroxyeicosatetraenoic acid (5-HETE) than control suspensions (P less than 0.05). Peripheral leucocytes, collected during attacks (n = 3) and studied without addition of A23187 released LTB4 in vitro but not sulphidopeptides leukotrienes. This result was never observed with leucocytes from control subjects or from the patient out of a crisis. These results suggest that in the patient, peripheral leucocytes could be stimulated by an unknown, as yet to be determined, endogenous factor to produce more 5-HETE and LTB4. Whether LTB4 plays a pathogenic role in the capillary leakage remains to be determined.
周期性系统性毛细血管渗漏综合征（克拉克森病）的特征是不明原因的毛细血管通透性显著增加发作。由于通过5-脂氧合酶途径从花生四烯酸衍生而来的白三烯会增强毛细血管通透性，我们研究了一名毛细血管渗漏综合征患者白细胞中的花生四烯酸代谢。在无症状期从患者采集的血液制备的白细胞-血小板悬液（n = 11）比对照悬液产生更多的5-羟基二十碳四烯酸（5-HETE）（P小于0.05）。在发作期间采集的外周白细胞（n = 3），在不添加A23187的情况下进行体外研究，可释放LTB4，但不释放硫肽白三烯。对照受试者或非发作期患者的白细胞从未观察到这一结果。这些结果表明，在该患者中，外周白细胞可能受到一种未知的、尚未确定的内源性因子刺激，从而产生更多的5-HETE和LTB4。LTB4是否在毛细血管渗漏中起致病作用仍有待确定。
