IMAGE-ANALYSIS STUDIES OF THE DEGREE OF IRREVERSIBLE DEFORMATION OF SICKLE CELLS IN RELATION TO CELL-DENSITY AND HB-F LEVEL
IMAGE-ANALYSIS STUDIES OF THE DEGREE OF IRREVERSIBLE DEFORMATION OF SICKLE CELLS IN RELATION TO CELL-DENSITY AND HB-F LEVEL
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DOI：
10.1111/j.1365-2141.1993.tb03179.x
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发表时间：
1993-10-01
影响因子：
6.5
通讯作者：
OHENEFREMPONG, K
中科院分区：
文献类型：
--
作者：
HORIUCHI, K;STEPHENS, MJ;OHENEFREMPONG, K
我们用图像分析法定量分析了纯合子镰状细胞病患者红细胞（SS细胞）的不可逆变形程度，并研究了不可逆变形程度、细胞密度和Hb F水平之间的关系。25例患者（年龄1-36岁）的SS细胞，Hb F含量为2.5%~ 40.0%。对各组分进行形态学观察和Hb F含量测定。我们发现，SS细胞从循环的不可逆变形发生主要是通过伸长和伸长的程度是非常可变的。我们还发现，在Hb F水平< 20%的患者的细胞中，不可逆伸长的程度随着细胞密度的增加而增加，这表明脱水伴随着不可逆伸长而发生。统计学分析（Student t检验）表明，HbF <20%患者SS细胞的伸长程度有显著性差异（P = 0.008或< 0.001），而HbF ≥ 20%患者SS细胞的伸长程度无显著性差异（P > 0.1）。在Hb F < 20%的患者中，细胞密度随Hb F浓度的降低而增加，但在Hb F ≥ 20%的患者中，细胞密度随Hb F浓度的降低而增加。这表明具有较低Hb F水平的细胞选择性地易受脱水影响。此外，我们发现，平均程度的不可逆延长线性下降，增加水平的血红蛋白F，并达到正常范围内的21- 24%。由于通过图像分析定量的SS细胞的不可逆变形的程度与细胞密度直接相关，并且与Hb F水平成反比，因此由Hb S聚合引起的机械应力或膜损伤可能是体内致密细胞形成的重要因素。
We analysed, quantitatively by image analysis, the degree of irreversible deformation of red cells (SS cells) from patients with homozygous sickle cell disease, and studied the relationships among the degree of irreversible cell deformation, cell density, and Hb F level. SS cells from 25 patients (aged 1-36 years) whose Hb F levels ranged from 2.5% to 40.0%, were fully oxygenated and then were separated into four fractions by density centrifugation. Every fraction was studied for morphology and Hb F content. We found that the irreversible deformation of SS cells from the circulation occurred mainly by elongation and that the degree of elongation was extremely variable. We also found that in the cells of patients with Hb F levels < 20% the degree of irreversible elongation increases as cell density increases, suggesting that dehydration occurs concomitantly with irreversible elongation. Statistical analysis (Student t test) indicated that there were significant differences (P = 0.008 or < 0.001) in the degree of elongation among density-fractionated SS cells from patients with Hb F < 20%, although there was no significant difference (P > 0.1) among those from patients with Hb F greater-than-or-equal-to 20%. We also found that cell density increased as Hb F level of the density fraction decreased in all patients with Hb F < 20% but not always in those with Hb F greater-than-or-equal-to 20%. This suggests that cells with lower Hb F levels are selectively susceptible to dehydration. Furthermore, we found that the mean degree of irreversible elongation decreases linearly with increasing levels of Hb F and reaches the normal range at 21-24%. Since the degree of irreversible deformation of SS cells quantified by image analysis is directly related to cell density, and inversely to Hb F levels, mechanical stress or membrane damage caused by Hb S polymerization may be an important factor in the formation of dense cells in vivo.