Tshilolo L, Summa V, Gregorj C, Kinsiama C, Bazeboso J A, Avvisati G, Labie D
Unité de Dépistage de la Drépanocytose, Centre Hospitalier Monkole, BP 817, Kinshasa XI, Democratic Republic of Congo.
Anemia. 2012;2012:105349. doi: 10.1155/2012/105349. Epub 2012 Jul 5.
High HbF levels and F cells are correlated with reduced morbidity and mortality in sickle cell disease (SCD). This paper was designed to determine the HbF and F cells levels in Congolese sickle cell anemia (SCA) patients in order to determine their impact on the expression of SCD. Population and Method. HbF levels were measured in 89 SCA patients (mean age 11.4 yrs) using a standard HPLC method. F cell quantitation was done in a second group of SCA patients (n = 42, mean age 8.9 yrs) and compared with a control group (n = 47, mean age 5 yrs). F cells were quantified by a cytofluorometric system (MoAb-HbF-FITC; cut off at 0.5%). Results. The mean value of HbF was 7.2% ± 5.0 with heterogeneous distribution, most patients (76%) having HbF < 8%. Mean values of F-cells in SCA patients and control group were 5.4% ± 7.6 (median: 2.19%; range 0,0-30,3%) and 0.5% ± 1.6 (median 0.0, range 0-5.18), respectively. SCA patients with F cells >4.5% developed less painful crisis and had higher percentage of reticulocytes. Conclusion. Congolese SCA patients displayed low levels of HbF and F-cells that contribute to the severity of SCD.
高血红蛋白F（HbF）水平和F细胞与镰状细胞病（SCD）发病率和死亡率的降低相关。本文旨在测定刚果镰状细胞贫血（SCA）患者的HbF和F细胞水平，以确定它们对SCD表达的影响。研究对象与方法。采用标准高效液相色谱法测定89例SCA患者（平均年龄11.4岁）的HbF水平。对另一组SCA患者（n = 42，平均年龄8.9岁）进行F细胞定量，并与对照组（n = 47，平均年龄5岁）进行比较。通过细胞荧光测定系统（单克隆抗体-HbF-异硫氰酸荧光素；临界值为0.5%）对F细胞进行定量。结果。HbF的平均值为7.2%±5.0%，分布不均一，大多数患者（76%）的HbF<8%。SCA患者和对照组F细胞的平均值分别为5.4%±7.6%（中位数：2.19%；范围0.0-30.3%）和0.5%±1.6%（中位数0.0，范围0-5.18）。F细胞>4.5%的SCA患者发生疼痛性危象的次数较少，网织红细胞百分比更高。结论。刚果SCA患者的HbF和F细胞水平较低，这导致了SCD的严重程度。
