Horiuchi K, Fynn-Thompson F, Ohene-Frempong K
Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104.
Exp Hematol. 1994 Oct;22(11):1058-63.
To study the effect of fetal hemoglobin (Hb F) on the formation of dense and irreversibly deformed cells in patients with sickle cell disease (SCD), we investigated the distribution of Hb F and Hb F cells (FC, cells having HbF) and the difference in the degree of irreversible deformation between FC and non-Hb F cells (NFC) in relation to their cell density. We used immunofluorescence to define FC and image analysis to quantify the degree of deformation. Blood samples from 16 patients with Hb F levels ranging from 4 to 24% were separated into less dense [top (T), d < 1.11 g/mL] and dense [bottom (B), d > or = 1.11 g/mL] fractions. We found higher percentages of Hb F and FC in top fractions, suggesting that FC are hydrated more than NFC. Quantitative shape analysis demonstrated that the irreversible deformation of FC in the top fraction [FC(T)] was not significantly different from that of NFC in the fraction [NFC(T)] (p = 0.163), but rather that the irreversible deformation of FC in the bottom fraction [FC(B)] was much milder than for NFC(B) (p < 0.001). The order of the degree of irreversible deformation was NFC(B) > FC(B) > NFC(T) = FC(T). These results clearly demonstrated that FC are less susceptible to both irreversible deformation and dehydration than NFC, suggesting that in circulation, the sickling process plays a major role in cell dehydration. Additionally, we found that the ratio of NFC(B) to FC(B) decreased as a patient's Hb F increased (r = 0.84) and that NFC(B) = FC(B) when Hb F was about 20%.
为研究胎儿血红蛋白（Hb F）对镰状细胞病（SCD）患者致密且不可逆变形细胞形成的影响，我们调查了Hb F和Hb F细胞（FC，即含有HbF的细胞）的分布，以及FC与非Hb F细胞（NFC）在不可逆变形程度上的差异与其细胞密度的关系。我们使用免疫荧光法界定FC，并通过图像分析量化变形程度。从16名Hb F水平在4%至24%之间的患者采集血样，分离为低密度[上层（T），d < 1.11 g/mL]和高密度[下层（B），d ≥ 1.11 g/mL]部分。我们发现上层部分中Hb F和FC的百分比更高，这表明FC比NFC水化程度更高。定量形状分析表明，上层部分的FC[FC(T)]的不可逆变形与该部分的NFC[NFC(T)]无显著差异（p = 0.163），但下层部分的FC[FC(B)]的不可逆变形比NFC(B)要温和得多（p < 0.001）。不可逆变形程度的顺序为NFC(B) > FC(B) > NFC(T) = FC(T)。这些结果清楚地表明，FC比NFC更不易发生不可逆变形和脱水，这表明在循环中，镰变过程在细胞脱水中起主要作用。此外，我们发现随着患者Hb F增加，NFC(B)与FC(B)的比例降低（r = 0.84），当Hb F约为20%时，NFC(B) = FC(B)。
