Kene-Udemezue Blessing E, Salako Abideen O, Akinsete Adeseye M, Odubela Oluwatosin O, Adeyemo Titilope A
Department of Paediatrics, Lagos University Teaching Hospital, Idi Araba, Lagos, Nigeria.
Clinical Sciences Department, Nigerian Institute of Medical Research, Yaba, Lagos, Nigeria.
Adv Hematol. 2024 Nov 23;2024:5002373. doi: 10.1155/ah/5002373. eCollection 2024.
Hydroxyurea (HU) is a disease-modifying therapy with significant clinical and laboratory efficacy among individuals living with sickle cell anaemia (SCA). This is evident through increased fetal haemoglobin, higher packed cell volume, improved red cell hydration, reduced leukocytes, and platelet function. The effect on the coagulation pathway and pathophysiologic mechanism remains unclear, especially in children living with SCA. This study evaluated the coagulation profile using D-dimer and thrombin antithrombin complex (TAT) in children with SCA. The cross-sectional study was conducted over three months at LUTH among 80 children living with SCA in steady state aged 2-18 years (40 HU exposed and 40 HU naïve, respectively). Blood samples were assayed for D-dimer, TAT, and complete blood count. Descriptive analysis such as mean and standard deviation for normally distributed variables or median and interquartile range for skewed data were used to summarize continuous variables, while proportion or percentages for categorical variables. Univariate analysis and bivariate analysis were done and statistical significance was set at < 0.05. The mean age (±SD) of study participants in both groups was 11.35 (±4.6 years). D-dimer levels (23.27 ng/mL) and TAT (29.79 pg/mL) were significantly lower among HU exposed compared to HU naïve children (62.73 ng/mL and 109.34 pg/mL, respectively) < 0.001. There was a negative correlation between D-dimer and TAT with the duration of HU use ( = -0.499, =0.001, and = -0.401, =0.010), respectively. There was a positive correlation between D-dimer and TAT with total WBC ( = 0.368, =0.019, and = 0.385, =0.014, respectively) among the HU naïve participants and a negative correlation between D-dimer and TAT with haemoglobin level ( = -0.303, =0.047, and = -0.311, =0.041, respectively) among HU exposed children. HU modulates the D-dimer and TAT levels of children living with SCA toward the normal reference range, thus reducing the risk of hypercoagulability and associated sequelae. Therefore, continuous advocacy for HU use should entail close monitoring of adverse effects.
羟基脲（HU）是一种改善病情的疗法，对镰状细胞贫血（SCA）患者具有显著的临床和实验室疗效。这一点从胎儿血红蛋白增加、红细胞压积升高、红细胞水合改善、白细胞减少以及血小板功能改善中可以明显看出。其对凝血途径和病理生理机制的影响仍不明确，尤其是在患有SCA的儿童中。本研究使用D - 二聚体和凝血酶抗凝血酶复合物（TAT）评估了SCA儿童的凝血情况。这项横断面研究在拉各斯大学教学医院（LUTH）进行了三个月，研究对象为80名年龄在2至18岁处于稳定状态的SCA儿童（分别有40名接触过HU和40名未接触过HU）。对血样进行了D - 二聚体、TAT和全血细胞计数检测。对于正态分布变量，使用均值和标准差等描述性分析来总结连续变量；对于偏态数据，则使用中位数和四分位数间距；对于分类变量，使用比例或百分比。进行了单因素分析和双因素分析，设定统计学显著性为<0.05。两组研究参与者的平均年龄（±标准差）为11.35（±4.6岁）。与未接触HU的儿童相比，接触HU的儿童中D - 二聚体水平（23.27 ng/mL）和TAT（29.79 pg/mL）显著更低（分别为62.73 ng/mL和109.34 pg/mL）<0.001。D - 二聚体和TAT与HU使用时长之间分别存在负相关（r = -0.499，p = 0.001，以及r = -0.401，p = 0.010）。在未接触HU的参与者中，D - 二聚体和TAT与白细胞总数之间存在正相关（分别为r = 0.368，p = 0.019，以及r = 0.385，p = 0.014）；在接触HU的儿童中，D - 二聚体和TAT与血红蛋白水平之间存在负相关（分别为r = -0.303，p = 0.047，以及r = -0.311，p = 0.041）。HU可将SCA儿童的D - 二聚体和TAT水平调节至正常参考范围，从而降低高凝风险及相关后遗症。因此，持续倡导使用HU应包括密切监测不良反应。
