Sickle Cell Clinic and Molecular Biology Laboratory, Odisha Sickle Cell Project (NHM), Veer Surendra Sai Institute of Medical Science and Research (VIMSAR), Burla, Sambalpur, Odisha, India.
Department of Bioscience and Biotechnology, Fakir Mohan University, Balasore, Odisha, India.
Ann Hematol. 2019 Oct;98(10):2257-2265. doi: 10.1007/s00277-019-03776-x. Epub 2019 Aug 22.
Vascular complications of sickle cell anemia (SCA) are influenced by many factors. Elevated plasma homocysteine (Hcy) is supposed to be an independent risk factor and is either genetic or nutritional origin. The present study evaluated the plasma Hcy level, MTHFR C677T gene polymorphism, effect of folic acid (FA) supplementation' and hemato-biochemical parameters in SCA and their effect on the vaso-occlusive crisis (VOC) in SCA patients of an Asian-Indian haplotype population. One hundred twenty cases of SCA (HbSS) and 50 controls with normal hemoglobin(HbAA) were studied. It was found that the plasma Hcy level is significantly higher (p < 0.0001) in patients with SCA (22.41 ± 7.8 μmol/L) compared to controls (13.2 ± 4.4 μmol/L). Moreover, patients without FA supplementation had a significantly (p < 0.001) higher Hcy level (27 ± 7 μmol/L) compared to those with supplementation (17.75 ± 5.7 μmol/L). Turkey-Kramer multiple comparison tests show that there is a significant difference (p < 0.05) in HbF percent, hemoglobin (Hb), platelet count, serum bilirubin (direct:Bil-D and total:Bil-T), aspartate transaminase (AST), lactate dehydrogenase (LDH), and plasma Hcy levels between mild and severe VOC. Between moderate VOC and severe VOC, there was a significant difference (p < 0.05) in HbF%, Bil-D, AST, Hcy. Pearson correlation revealed that plasma Hcy had a significantly (p < 0.05) positive correlation with AST, serum bilirubin (indirect and total), LDH, jaundice, stroke, VOC per year, and hospitalization per year whereas it was inversely correlated with HbF percentage, Hb level, and FA treatment. In the study population, increased plasma Hcy level, hemolysis, and platelet activation were found to influence VOC in SCA.
镰状细胞贫血（SCA）的血管并发症受多种因素影响。升高的血浆同型半胱氨酸（Hcy）被认为是一个独立的危险因素，其来源既有遗传的也有营养的。本研究评估了血浆 Hcy 水平、MTHFR C677T 基因多态性、叶酸（FA）补充的效果以及血液生化参数在 SCA 中的变化及其对 SCA 患者血管阻塞性危象（VOC）的影响。研究了 120 例 SCA（HbSS）和 50 例正常血红蛋白（HbAA）对照者。结果发现，SCA 患者的血浆 Hcy 水平明显高于对照组（22.41±7.8μmol/L 比 13.2±4.4μmol/L，p<0.0001）。此外，未补充 FA 的患者 Hcy 水平显著升高（p<0.001，27±7μmol/L），而补充 FA 的患者 Hcy 水平（17.75±5.7μmol/L）较低。Turkey-Kramer 多重比较检验显示，在轻度和重度 VOC 之间，HbF 百分率、血红蛋白（Hb）、血小板计数、血清胆红素（直接：Bil-D 和总：Bil-T）、天冬氨酸转氨酶（AST）、乳酸脱氢酶（LDH）和血浆 Hcy 水平有显著差异（p<0.05）。在中度 VOC 和重度 VOC 之间，HbF%、Bil-D、AST、Hcy 有显著差异（p<0.05）。Pearson 相关性分析显示，血浆 Hcy 与 AST、血清胆红素（间接和总）、LDH、黄疸、中风、每年 VOC 发作次数和每年住院次数呈显著正相关（p<0.05），与 HbF 百分率、Hb 水平和 FA 治疗呈负相关。在研究人群中，发现血浆 Hcy 水平升高、溶血和血小板激活影响 SCA 中的 VOC。
