Foetal haemoglobin, erythrocytes containing foetal haemoglobin, and hematological features in congolese patients with sickle cell anaemia.
Foetal haemoglobin, erythrocytes containing foetal haemoglobin, and hematological features in congolese patients with sickle cell anaemia.
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作者：
Tshilolo L;Summa V;Gregorj C;Kinsiama C;Bazeboso JA;Avvisati G;Labie D
高HbF水平和F细胞与镰状细胞病（SCD）发病率和死亡率降低相关。本文旨在检测刚果镰状细胞性贫血（SCA）患者的HbF和F细胞水平，以确定其对SCD表达的影响。人口与方法。使用标准HPLC法测量89例SCA患者（平均年龄11.4岁）的HbF水平。在第二组SCA患者（n = 42，平均年龄8.9岁）中进行F细胞定量，并与对照组（n = 47，平均年龄5岁）进行比较。通过细胞荧光系统（MoAb-HbF-FITC；在0.5%时切断）对F细胞进行定量。结果。HbF平均值为7.2%±5.0，呈非均匀分布，多数患者（76%）HbF < 8%。SCA患者和对照组的f细胞平均值分别为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）。含有F细胞4.5%的SCA患者出现较少的疼痛危象，网状红细胞比例较高。结论。刚果SCA患者表现出低水平的HbF和f细胞，这有助于SCD的严重程度。
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.