Effect of Bone Pain Vaso-occlusive Crises on Red Blood Cell Parameters of Sickle Cell Anaemia Patients

A. Musa, M. Ndakotsu
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Abstract

Background: Sickle Cell Anaemia (SCA) is an inherited haemoglobin disorder characterized by shortened red cell life span and altered red blood cell parameters due to the red cell sickling, vaso-occlusion and chronic haemolysis occasioned by the disease. The occurrence of bone pain vaso-occlusive crises may further alter these parameters; an occurrence that highlights on the pathophysiology of SCA and worth noting in the management of affected patients. Aims and Objectives: This study was performed to determine the effect of bone pain vaso-occlusive crises on red blood cell parameters of SCA patients. Study Design: A longitudinal study. Settings: Fifty SCA patients receiving care at a tertiary hospital in Sokoto, Nigeria were consecutively enrolled while in their steady clinical state and followed up till when they re-presented during the bone pain vaso-occlusive crises state. Materials and Methods: Structured proforma was used for clinical data capturing while red blood cell parameters (red blood cell count (RBC), haemoglobin (Hb) concentration, haematocrit (HCT), mean corpuscular volume (MCV), mean corpuscular haemoglobin (MCH), mean corpuscular haemoglobin concentration (MCHC) and red cell distribution width (RDW) were determined via automation using Nortek 3-part haematology analyser. Leishmann stained peripheral blood smears were examined to cross-check morphological details of the red blood cells. Statistics: Data analysis was performed using Statistical Package for the Social Sciences (SPSS) version 21.0 (IBM Corp, Armonk, NY, USA) and further summarized as means ± standard deviations. Comparison of means was performed using paired sample t-test and Anova as appropriate while Pearson’s test was used for correlation analysis. Results were presented in tables while statistical significance was set at p<0.05. Results: Fifty SCA patients were enrolled for the study and consisted of 26 (52%) females and 24 (48%) males. The overall mean age of the study participants was 22.82 ± 6.55 years with the minimum and maximum ages of 18 and 42 respectively. During the bone pain vaso-occlusive crises, 14 (28%), 21 (42%) and 15 (30%) of the patients had mild, moderate and severe bone pains respectively. The red blood cell parameters during the steady and bone pain crises states were RBC count (2.87±0.69 vs. 2.93±0.83; p=0.675); HCT (24.33±5.01 vs. .37±5.61; p=0.373); Hb concentration (8.34±1.53 vs. 7.99±1.54; p=0.228); MCV (87.87±9.13 vs. 82.59±14.64; p=0.021); MCH (29.76±5.61 vs. 30.48±13.54; p =0.729); MCHC (32.89±4.76 vs. 32.10±3.75; p=0.394) and RDW-CV (19.30±6.58 vs. 19.59±5.27; p=0.829) respectively. During bone pain vaso-occlusive crises, the red blood cell parameters based on mild, moderate and severe pains were RBC count (2.82±0.88 vs. 2.78±0.57 and 3.06±0.65; p=0.472); HCT (24.29±6.37 vs. 24.11±5.12 and 24.68±3.53; p=0.947); Hb concentration (8.46±2.01 vs. 8.33±1.57 and 8.24±0.92; p=0.927); MCV (87.60±7.46 vs. 91.03±9.21 and 83.69±9.22, p=0.055); MCH (31.28±7.77 vs. 30.27±4.27 and 27.61±4.51; p=0.185); MCHC (33.05±7.54 vs. 33.07±3.39 and 32.48±3.13; p=0.928) and RDW-CV (19.61±9.65 vs. 19.96±5.61 and 18.10±4.23; p=0.700) respectively. Conclusions: Of all the studied red cell parameters, only MCV significantly differ between the steady and bone pain crises states of SCA. A drop in MCV was recorded during the bone pain crises; probably reflecting depletion of the large reticulocytes and neocytes during the accentuated red cell sickling and vaso-occlusive events characteristic of this clinical state.
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骨痛血管闭塞性危象对镰状细胞贫血患者红细胞参数的影响
背景:镰状细胞性贫血(SCA)是一种遗传性血红蛋白疾病,其特征是由于该病引起的红细胞镰状坏死、血管闭塞和慢性溶血导致红细胞寿命缩短和红细胞参数改变。骨痛血管闭塞危象的发生可能进一步改变这些参数;这一事件突出了SCA的病理生理学,在受影响患者的管理中值得注意。目的和目的:本研究旨在确定骨痛血管闭塞危象对SCA患者红细胞参数的影响。研究设计:纵向研究。背景:在尼日利亚索科托一家三级医院接受治疗的50例SCA患者在其稳定的临床状态下连续入组,并随访至他们在骨痛血管闭塞危象状态下再次出现。材料和方法:临床数据采集采用结构化形式,红细胞参数(红细胞计数(RBC)、血红蛋白(Hb)浓度、红细胞压积(HCT)、平均红细胞体积(MCV)、平均红细胞血红蛋白(MCH)、平均红细胞血红蛋白浓度(MCHC)和红细胞分布宽度(RDW)由Nortek三部分血液学分析仪自动化测定。外周血Leishmann染色涂片交叉检查红细胞的形态学细节。统计学:使用SPSS 21.0版(IBM Corp, Armonk, NY, USA)进行数据分析,并进一步总结为平均值±标准差。采用配对样本t检验和方差分析进行均数比较,相关分析采用Pearson检验。结果以表格形式呈现,p<0.05为有统计学意义。结果:50例SCA患者入组研究,其中女性26例(52%),男性24例(48%)。研究参与者的总体平均年龄为22.82±6.55岁,最小年龄为18岁,最大年龄为42岁。在骨痛血管闭塞危象期间,轻度、中度和重度骨痛分别为14例(28%)、21例(42%)和15例(30%)。稳定状态和骨痛危象状态的红细胞参数为:红细胞计数(2.87±0.69∶2.93±0.83);p = 0.675);HCT(24.33±5.01∶0.37±5.61);p = 0.373);Hb浓度(8.34±1.53∶7.99±1.54);p = 0.228);MCV(87.87±9.13∶82.59±14.64);p = 0.021);MCH(29.76±5.61∶30.48±13.54);p = 0.729);MCHC(32.89±4.76∶32.10±3.75);p=0.394)和RDW-CV(19.30±6.58∶19.59±5.27;分别p = 0.829)。在骨痛血管闭塞危象时,基于轻度、中度和重度疼痛的红细胞参数为RBC计数(2.82±0.88比2.78±0.57和3.06±0.65);p = 0.472);HCT(24.29±6.37 vs. 24.11±5.12和24.68±3.53);p = 0.947);Hb浓度(8.46±2.01∶8.33±1.57∶8.24±0.92);p = 0.927);MCV(87.60±7.46 vs. 91.03±9.21和83.69±9.22,p=0.055);MCH(31.28±7.77∶30.27±4.27∶27.61±4.51);p = 0.185);MCHC(33.05±7.54∶33.07±3.39∶32.48±3.13);p=0.928)和RDW-CV(19.61±9.65 vs. 19.96±5.61和18.10±4.23;分别p = 0.700)。结论:在所有研究的红细胞参数中,只有MCV在SCA稳定状态和骨痛危象状态之间存在显著差异。在骨痛危机期间,MCV记录下降;可能反映了在这种临床状态的红细胞镰状增生和血管闭塞事件中大网织红细胞和新生细胞的耗竭。
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