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加蓬东部农村地区稳态镰状细胞贫血患者及匹配的血红蛋白AA对照者的血液学值

Haematological values in steady-state sickle cell anaemia patients and matched heamoglobin AA Controls in a Rural Area of Eastern Gabon.

作者信息

Mombo Landry Erik, Mabioko-Mbembo Gaël, Bisseye Cyrille, Mbacky Kevin, Thiam Fatoumata, Edou Apollinaire

机构信息

Laboratory of Molecular and Cellular Biology (LABMC), University of Science and Technology of Masuku (USTM), Franceville, Gabon.

Laboratory of Molecular and Cellular Biology (LABMC), University of Science and Technology of Masuku (USTM), Franceville; Paul Moukambi Regional Hospital Centre of Koula-Moutou (CHRPM), Koula-Moutou, Gabon.

出版信息

Niger Postgrad Med J. 2019 Jan-Mar;26(1):13-17. doi: 10.4103/npmj.npmj_182_18.

DOI:10.4103/npmj.npmj_182_18
PMID:30860194
Abstract

BACKGROUND

In Gabon, universal neonatal screening of sickle cell disease is not carried out in rural areas, often leading to late detection of the disease. However, complete blood counts are available in rural areas.

MATERIALS AND METHODS

We evaluated the haematological parameters of 45 homozygous steady-state sickle cell anaemia (SCA) patients and compared them with 45 sex- and age-matched Haemoglobin AA controls in Koula-Moutou, a rural area in Eastern Gabon.

RESULTS

Homozygous SCA patients had low erythrocyte values (red blood cells: 2.50 × 10/L, haemoglobin: 7.20 g/dL and haematocrit: 20.70%) and high leucocyte values (white blood cells: 14.40 × 10/L, lymphocytes: 5.24 × 10/L and monocytes: 1.60 × 10/L). Most of the SCA patients had severe anaemia (67%), normochromia (76%), lymphocytosis (73%) and monocytosis (84%). A haemoglobin level of < 8.5 g/dL together with a leucocyte level above 9.5 × 10 cells/L was used as screening test to detect homozygous SCA patients, with sensitivity of 84.4% and specificity of 97.8%.

CONCLUSION

The values for erythrocyte and leucocyte cell lines of SCA patients in steady state are clearly different from those of the matched HbA/A controls. This makes it possible to set up a tool to detect SCA based on the haemogram in a rural area that does not possess haemoglobin electrophoresis. This tool could be used by healthcare workers in the absence of universal newborn screening for SCA.

摘要

背景

在加蓬,农村地区未开展镰状细胞病的新生儿普遍筛查,这常常导致该病的检测延迟。然而,农村地区可进行全血细胞计数。

材料与方法

我们评估了加蓬东部农村地区库拉穆图45例纯合子稳态镰状细胞贫血(SCA)患者的血液学参数,并将其与45例性别和年龄匹配的血红蛋白AA对照者进行比较。

结果

纯合子SCA患者红细胞值较低(红细胞:2.50×10/L,血红蛋白:7.20 g/dL,血细胞比容:20.70%),白细胞值较高(白细胞:14.40×10/L,淋巴细胞:5.24×10/L,单核细胞:1.60×10/L)。大多数SCA患者有严重贫血(67%)、正常色素性贫血(76%)、淋巴细胞增多(73%)和单核细胞增多(84%)。血红蛋白水平<8.5 g/dL且白细胞水平高于9.5×10细胞/L被用作筛查试验以检测纯合子SCA患者,敏感性为84.4%,特异性为97.8%。

结论

稳态SCA患者的红细胞和白细胞系数值与匹配的HbA/A对照者明显不同。这使得在没有血红蛋白电泳的农村地区有可能建立一种基于血常规检测SCA的工具。在没有SCA新生儿普遍筛查的情况下,医护人员可使用该工具。

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