Department of Hematology, Edith Wolfson Medical Center, Holon, Israel.
Ann Hematol. 2011 May;90(5):509-13. doi: 10.1007/s00277-010-1110-2. Epub 2010 Nov 10.
In the present study, we questioned the role of oxidative stress in hereditary spherocytosis (HS), where red blood cells (RBC) have a shortened survival due to primary deficiency in membrane proteins. Using flow cytometry techniques, we showed that RBC derived from 17 HS patients of seven families generate more reactive oxygen species, membrane lipid peroxides, and less reduced glutathione than normal RBC. Following in vitro incubation of HS-RBC from seven patients with a fermentation bioproduct of Carica papaya (fermented papaya preparation (FPP)) with known antioxidative properties, oxidative stress markers were significantly reduced. Similar results were obtained following treatment with FPP for 3 months of 10 adult HS patients, as well as decreased tendency to undergo hemolysis. The hemoglobin levels increased by >1 g/dl, mean corpuscular hemoglobin concentration decreased by >1 g/dl, and the reticulocyte count decreased by 0.93%. Concomitantly, lactic dehydrogenase decreased by 17% and indirect bilirubin by 50%. A significant decrease in malonyldialdehyde was also detected. These data indicate that oxidative stress plays an important role in the pathophysiology of HS which can be ameliorated by an antioxidant such as FPP. Additional clinical trials with FPP and other antioxidants are warranted.
在本研究中，我们探讨了氧化应激在遗传性球形红细胞增多症（HS）中的作用，HS 患者由于膜蛋白的主要缺陷导致红细胞（RBC）寿命缩短。我们使用流式细胞术技术表明，来自七个家族的 17 名 HS 患者的 RBC 产生的活性氧、膜脂质过氧化物比正常 RBC 更多，而还原型谷胱甘肽则更少。对来自 7 名患者的 HS-RBC 进行体外孵育，用具有已知抗氧化特性的木瓜发酵生物制品（发酵木瓜制剂（FPP））孵育后，氧化应激标志物显著降低。对 10 名成年 HS 患者用 FPP 治疗 3 个月，也得到了类似的结果，同时还降低了溶血的趋势。血红蛋白水平升高>1g/dl，平均红细胞血红蛋白浓度升高>1g/dl，网织红细胞计数降低 0.93%。同时，乳酸脱氢酶降低 17%，间接胆红素降低 50%。还检测到丙二醛显著减少。这些数据表明氧化应激在 HS 的病理生理学中起重要作用，抗氧化剂如 FPP 可改善这种情况。需要进行更多的临床试验，以评估 FPP 和其他抗氧化剂的疗效。
