Billett H H, Kim K, Fabry M E, Nagel R L
Blood. 1986 Jul;68(1):301-3.
To test the hypothesis that the tendency of hemoglobin S (HbS) to polymerize within cells is the major determinant of the incidence of vaso-occlusive episodes, we have examined the effect of the percentage of dense cells (as measured by Percoll-Stractan continuous density gradient centrifugation) on the frequency of painful crises in a group of 36 patients with sickle cell disease. No correlation was found between the percentage of dense cells and admissions for crisis. Among the patients with known alpha-gene status (n = 25), the strong correlation between decreased dense cells and alpha-thalassemia (-alpha/alpha alpha) reported previously was confirmed (P less than .001). In addition, in this small subset, patients with alpha-thalassemia (-alpha/alpha alpha) appeared to have a marginally increased number of admissions for sickle cell crisis (t = 2.1910, P less than .05), which was independent of the percentage of dense cells. We conclude that the percentage of dense sickle cells cannot predict the incidence of painful crisis, suggesting that other factors (microcirculatory regulation or other humoral and cellular factors) are more important in the generation maintenance of painful crises than the necessary, but not sufficient, tendency of HbS-containing red cells to sickle.
为了验证血红蛋白S(HbS)在细胞内聚合的倾向是血管阻塞性发作发生率的主要决定因素这一假说,我们研究了致密细胞百分比(通过Percoll-Stractan连续密度梯度离心法测量)对一组36例镰状细胞病患者疼痛性危象发生频率的影响。未发现致密细胞百分比与因危象入院之间存在相关性。在已知α基因状态的患者(n = 25)中,先前报道的致密细胞减少与α地中海贫血(-α/αα)之间的强相关性得到了证实(P <.001)。此外,在这个小亚组中,α地中海贫血(-α/αα)患者因镰状细胞危象入院的次数似乎略有增加(t = 2.1910,P <.05),这与致密细胞百分比无关。我们得出结论,致密镰状细胞的百分比不能预测疼痛性危象的发生率,这表明在疼痛性危象的发生和维持中,其他因素(微循环调节或其他体液和细胞因素)比含HbS的红细胞镰变的必要但不充分的倾向更重要。