Ajuwon Mauryne Debola, Olayemi Edeghonghon, Benneh Amma Anima
Department of Medical Laboratory Technology, School of Allied Health Sciences University of Ghana, Accra, Ghana.
Ghana Institute of Clinical Genetics and Department of Haematology, University of Ghana, Accra, Ghana.
Pan Afr Med J. 2014 Nov 17;19:289. doi: 10.11604/pamj.2014.19.289.4451. eCollection 2014.
Sickle cell disease is a collection of autosomal recessive genetic disorders. It includes homozygous HbSS and double heterozygote combinations such as HbSC. Central and West Africa bears a significant burden of HbSC disease.
Prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen concentration (FC) and platelet count (PC) were determined in 41 HbSC and HbSS patients in steady state along with 40 apparently healthy HbAA controls. One way ANOVA test was used to compare means; p values< 0.05 were considered statistically significant.
There was no significant difference in mean PT for the study groups (p = 0.192). Mean PC was highest in HbSS patients: 445.7 +/- 128.3 X 10 9/ L compared to HbSC: 330.0 +/- 97.7 X 10 9/ L and HbAA:245 +/- 77.7 X 10 9/ L (p = 0.000). Mean APTT was 28.1 +/- 3.8 seconds in controls,24.1 + /- 66 seconds in HbSS patients and 21.8 +/- 3.8 seconds in HbSC patients (p = 0.000). Mean FC in HbSS was 1.6 +/- 0.7 g/L, 3.2 +/- 0.6 g/L in HbSC and 2.9 +/- 0.4 g/L in HbAA (p = 0.000).
A significant difference exists in PC, APTT and FC in HbSC patients compared to HbSS patients and HbAA controls. Elevated FC and shortened APTT may play a role in complications more characteristic of HbSC such as retinopathy and osteonecrosis. These suggest HbSC is not merely a milder form of HbSS; both diseases should be seen as different entities with regards to approaches for management.
镰状细胞病是一组常染色体隐性遗传疾病。它包括纯合子HbSS以及双杂合子组合，如HbSC。中非和西非地区HbSC疾病负担较重。
对41例处于稳定期的HbSC和HbSS患者以及40例表面健康的HbAA对照者测定凝血酶原时间（PT）、活化部分凝血活酶时间（APTT）、纤维蛋白原浓度（FC）和血小板计数（PC）。采用单因素方差分析比较均值；p值<0.05被认为具有统计学意义。
研究组间平均PT无显著差异（p = 0.192）。HbSS患者的平均PC最高：445.7±128.3×10⁹/L，而HbSC患者为330.0±97.7×10⁹/L，HbAA对照者为245±77.7×10⁹/L（p = 0.000）。对照组的平均APTT为28.1±3.8秒，HbSS患者为24.1±6.6秒，HbSC患者为21.8±3.8秒（p = 0.000）。HbSS患者的平均FC为1.6±0.7 g/L，HbSC患者为3.2±0.6 g/L，HbAA对照者为2.9±0.4 g/L（p = 0.000）。
与HbSS患者和HbAA对照者相比，HbSC患者的PC、APTT和FC存在显著差异。FC升高和APTT缩短可能在HbSC更具特征性的并发症如视网膜病变和骨坏死中起作用。这表明HbSC不仅仅是HbSS的一种较轻形式；就管理方法而言，这两种疾病应被视为不同的实体。
