Thomson A P, Dick M
Department of Paediatrics, St Thomas's Hospital Medical School, London.
Clin Lab Haematol. 1988;10(4):397-401. doi: 10.1111/j.1365-2257.1988.tb01187.x.
Fifty-nine children with sickle cell anaemia (HbSS) or associated haemoglobinopathies were studied prospectively using a chromogenic Limulus amoebocyte lysate assay to detect circulating endotoxin. The 41 children with HbSS (mean age 8 years 9 months) had more serious disease than the 18 with HbSC disease (n = 14) or HbS-beta-thalassaemia (n = 4) (mean age 7 years 2 months), with a greater degree of splenomegaly, lower haemoglobin, and higher white cell counts, platelet counts and bilirubin values (P less than 0.05 for all). Twenty-nine children with HbSS had evidence of poor reticuloendothelial function, with red cell pitting of greater than or equal to 2%. Three of these 29 had low levels of endotoxin in plasma (0.12-0.24 endotoxin units (EU)/ml); two were clinically well, one had a painful crisis. Eight of 18 children with other sickle haemoglobinopathies had greater than or equal to 2% pitted red cells; none was endotoxinaemic. Therefore, in 37 patients with reticuloendothelial dysfunction, three were endotoxinaemic; all had sickle cell anaemia. Although not statistically significant, this suggests that endotoxinaemia may occur predominantly in patients with reticuloendothelial dysfunction, and is compatible with the hypothesis that systemic endotoxinaemia can derive from the intestine especially when reticuloendothelial function is depressed.
采用显色鲎试剂法检测循环内毒素，对59例镰状细胞贫血（HbSS）或相关血红蛋白病患儿进行了前瞻性研究。41例HbSS患儿（平均年龄8岁9个月）比18例HbSC病（n = 14）或HbS-β地中海贫血（n = 4）患儿（平均年龄7岁2个月）病情更严重，脾肿大程度更高，血红蛋白水平更低，白细胞计数、血小板计数和胆红素值更高（所有P均小于0.05）。29例HbSS患儿有网状内皮系统功能不良的证据，红细胞凹陷率大于或等于2%。这29例中的3例血浆内毒素水平较低（0.12 - 0.24内毒素单位（EU）/ml）；2例临床状况良好，1例发生疼痛性危象。18例患有其他镰状血红蛋白病的患儿中有8例红细胞凹陷率大于或等于2%；均无内毒素血症。因此，在37例网状内皮系统功能障碍患者中，3例有内毒素血症；均为镰状细胞贫血。虽然无统计学意义，但这表明内毒素血症可能主要发生在网状内皮系统功能障碍的患者中，这与全身内毒素血症可源于肠道尤其是网状内皮系统功能低下时的假说相符。
