Kusfa I U, Mamman A I, Aminu S M, Hassan A, Muktar H M
Department of Haematology and Blood Transfusion, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria.
Niger J Clin Pract. 2017 Aug;20(8):998-1001. doi: 10.4103/njcp.njcp_363_16.
Alterations in the components of hemostasis, namely platelet function, the procoagulant, anticoagulant, and the fibrinolytic systems, are observed in sickle cell anemia (SCA) and are in favor of a procoagulant phenotype. Therefore, study of protein C and antithrombin (AT) levels in patients with SCA in steady state may be used in the treatment and/or prevention of SCA-related thrombotic complications. We studied the changes of these naturally occurring anticoagulants in patients with SCA attending the sickle cell clinic in Ahmadu Bello University Teaching Hospital, Zaria.
We conducted a case-control study involving 50 SCA (HbSS) patients in the steady state as cases and 25 healthy volunteers with normal hemoglobin (HbAA) as controls. Protein C and AT levels were estimated by semi-automation using Diagnostica Stago hematology coagulation analyzer. Frequencies, proportions, and independent t test were performed using SPSS version 20.
The mean ages of both the patients and controls were 23.80 ± 7.46 and 24.28 ± 3.48 years, respectively, and study participants comprised 40 (53.0%) women between the ages of 15-50 years and 15-34 years (P = 0.76). The mean values of protein C and AT levels in patients with SCA in the steady state and the control group were 60.26 ± 20.58% versus 81.30 ± 19.74%, 95% CI 11.13-30.96, and 42.11 ± 5.01% versus 61.88 ± 11.27%, 95% CI 16.03-23.51 with P values (P < 0.001), respectively.
This study showed that there was a significant decrease in the levels of protein C and AT between the SCA patients in the steady state and the controls. We recommend baseline investigations of these naturally occurring anticoagulants in patients with SCA, especially in those with frequent vaso-occlusive crises. This will give us an insight into the additional pathophysiologic mechanism in SCA-related thrombotic complications for better patient management and outcome.
在镰状细胞贫血（SCA）患者中观察到止血成分的改变，即血小板功能、促凝、抗凝和纤溶系统的改变，这些改变有利于促凝表型。因此，研究稳态SCA患者的蛋白C和抗凝血酶（AT）水平可能有助于治疗和/或预防SCA相关的血栓并发症。我们研究了在扎里亚阿马杜·贝洛大学教学医院镰状细胞门诊就诊的SCA患者中这些天然抗凝剂的变化。
我们进行了一项病例对照研究，纳入50例稳态SCA（HbSS）患者作为病例组，25例血红蛋白正常（HbAA）的健康志愿者作为对照组。使用Diagnostica Stago血液学凝血分析仪通过半自动方法测定蛋白C和AT水平。使用SPSS 20版进行频率、比例和独立t检验。
患者和对照组的平均年龄分别为23.80±7.46岁和24.28±3.48岁，研究参与者包括40名（53.0%）年龄在15 - 50岁和15 - 34岁之间的女性（P = 0.76）。稳态SCA患者和对照组的蛋白C和AT水平平均值分别为60.26±20.58%对81.30±19.74%，95%CI为11.13 - 30.96，以及42.11±5.01%对61.88±11.27%，95%CI为16.03 - 23.51，P值分别为（P < 0.001）。
本研究表明，稳态SCA患者与对照组相比，蛋白C和AT水平显著降低。我们建议对SCA患者，尤其是那些频繁发生血管闭塞性危机的患者，进行这些天然抗凝剂的基线调查。这将使我们深入了解SCA相关血栓并发症的额外病理生理机制，以更好地管理患者并改善预后。
