Powars D R, Schroeder W A, Weiss J N, Chan L S, Azen S P
J Clin Invest. 1980 Mar;65(3):732-40. doi: 10.1172/JCI109720.
Persons with sickle cell anemia who have elevated fetal hemoglobin or lowered erythrocyte mean corpuscular volume are reputed to have less severe clinical manifestations and a greater probability of survival. This study examines the relationship between seven clinical indicators of morbidity in sickle cell anemia and seven hematological parameters that were collected from 214 patients. Risks of sickle cell crisis, acute chest syndrome, hospital admissions, cerebrovascular accident, aseptic necrosis, meningitis/septicemia, and death were used as indicators of morbidity. The hematological parameters included percent fetal hemoglobin, absolute fetal hemoglobin, percent hemoglobin A2, hemoglobin concentration, packed cell volume, mean corpuscular volume, and mean corpuscular hemoglobin concentration. Statistical analyses of the data showed no relationship between the hematological parameters and six of the seven clinical indicators of the severity of sickle cell anemia. The only significant finding was an increased risk of stroke in those patients with lower levels of fetal hemoglobin. Therefore, with this exception, there is no predictable relationship between morbidity and mortality in sickle cell anemia and levels of fetal hemoglobin or erythrocyte indices. Thus, the general belief that there is an association between severity of sickle cell anemia and the levels of fetal hemoglobin has not been established.
患有镰状细胞贫血且胎儿血红蛋白升高或红细胞平均体积降低的人，据说其临床表现较轻，存活概率更大。本研究调查了从214名患者身上收集的镰状细胞贫血的七个发病临床指标与七个血液学参数之间的关系。镰状细胞危象、急性胸部综合征、住院、脑血管意外、无菌性坏死、脑膜炎/败血症和死亡的风险被用作发病指标。血液学参数包括胎儿血红蛋白百分比、绝对胎儿血红蛋白、血红蛋白A2百分比、血红蛋白浓度、红细胞压积、平均红细胞体积和平均红细胞血红蛋白浓度。数据的统计分析表明，血液学参数与镰状细胞贫血严重程度的七个临床指标中的六个没有关系。唯一显著的发现是胎儿血红蛋白水平较低的患者中风风险增加。因此，除了这个例外，镰状细胞贫血的发病率和死亡率与胎儿血红蛋白水平或红细胞指数之间没有可预测的关系。因此，镰状细胞贫血的严重程度与胎儿血红蛋白水平之间存在关联这一普遍观点尚未得到证实。
