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Abstract：
Sickle cell disease (SCD) is the most common monogenic disorder, although the diversity and heterogenicity of clinical presentations render estimations of disease severity unpredictable. This cross-sectional study aimed to determine if laboratory markers could serve as indicators of SCD severity. We enrolled 90 adult patients with SCD with a mean age of 32.33 ± 11.84 years from the eastern province of Saudi Arabia, where SCD is more common than in other regions. Our study revealed a positive significant association between the number of hospitalizations and emergency visits with white blood cells (WBC) (R = 0.241, R = 0.207), respectively. Similarly, positive significant associations were found between the number of hospitalizations and emergency visits with platelets (R = 0.393, R = 0.276), respectively. Conversely, negative significant relationships were found between the number of hospitalizations and emergency visits (ER) with hemoglobin (Hb) F (R = -0.268, R = -0.263), respectively. Additionally, significant negative relationships were found between Hb F (R = -0.223) and the frequency of ICU admission. Only the number of hospitalizations and emergency visits annually were significantly predicted with P values of 0.021 and 0.038, respectively. Moreover, an increase in WBC was found to significantly increase the chance of undergoing splenectomy by 23.02%. SCD is a multisystemic disease with heterogeneous clinical presentations and disease severity. Inflammatory markers are valuable tools for better risk stratification and could be translated into developing new therapeutic strategies and modifying the treatment paradigm.
摘要：
镰状细胞病（SCD）是最常见的单基因疾病，尽管临床表现的多样性和异质性使得疾病严重程度的估计无法预测。这项横断面研究旨在确定实验室标志物是否可以作为SCD严重程度的指标。我们招募了90名来自沙特阿拉伯东部省份的SCD成年患者，平均年龄为32.33±11.84岁。其中SCD比其他地区更常见。我们的研究表明，住院次数与白细胞（WBC）急诊次数之间存在正相关（R=0.241，R=0.207），分别。同样，发现住院次数与血小板急诊就诊次数之间存在显著的正相关(R=0.393,R=0.276),分别。相反，发现住院次数和急诊就诊（ER）与血红蛋白（Hb）F（R=-0.268，R=-0.263）之间存在负相关，分别。此外,HbF（R=-0.223）与ICU入院频率之间存在显着负相关。只有每年住院和急诊就诊的次数被显著预测，P值分别为0.021和0.038。此外，发现WBC的增加使行脾切除术的机会显著增加23.02%.SCD是一种多系统疾病，临床表现和疾病严重程度不同。炎症标志物是更好的风险分层的有价值的工具，可以转化为开发新的治疗策略和修改治疗范式。
