Jit Bimal Prasad, Mohanty Pradeep Kumar, Purohit Prasanta, Das Kishalaya, Patel Siris, Meher Satyabrata, Mohanty Jyoti Ranjan, Sinha Shalini, Behera Rajendra Kumar, Das Padmalaya
School of Life Sciences, AIPH University, Bhubaneswar, Odisha, India; Sickle Cell Clinic and Molecular Biology Laboratory, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India.
Sickle Cell Clinic and Molecular Biology Laboratory, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India; Department of Medicine, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur, Odisha, India.
Blood Cells Mol Dis. 2019 Mar;75:30-34. doi: 10.1016/j.bcmd.2018.12.003. Epub 2018 Dec 20.
Sickle cell disease (SCD) is a Mendelian single gene disorder with highly variable phenotypic expression. In the present study, we analyzed the influence of HbF, alpha thalassemia and other hematological indices to determine their association with acute pain episodes.
This case control study consisted of SCD subjects with HbS phenotype experiencing three or more acute pain episodes in last twelve months (cases) and without any episode of acute pain during last twelve months (controls). Hematological parameters, HbF, and presence of alpha thalassemia were assessed in all subjects.
A statistically significant difference between HbF levels (P < 0.025, χ2 test) and alpha thalassemia (P < 0.008, χ2 test) was observed between controls and cases group. Univariate analysis indicated that increased HbF levels > 25% (OR: 0.37, 95% CI: 0.18-0.77, P < 0.008) and presence of alpha thalassemia (OR: 0.53, 95% CI: 0.33-0.85, P < 0.009) provided protection, while multivariate analysis revealed significant protection was attributable only by higher HbF levels (OR: 0.39, 95% CI: 0.17-0.88, P < 0.025). Significantly higher HbF levels were observed only in the 11-20 age group of cases in comparison to controls (Student's t-test, P < 0.001).
Higher concentrations of HbF are associated with protection against frequent episodes of acute pain crisis in SCD patients.
镰状细胞病(SCD)是一种孟德尔单基因疾病,其表型表达高度可变。在本研究中,我们分析了胎儿血红蛋白(HbF)、α地中海贫血和其他血液学指标的影响,以确定它们与急性疼痛发作的关联。
本病例对照研究包括具有血红蛋白S(HbS)表型的SCD受试者,其中在过去十二个月内经历三次或更多次急性疼痛发作的为病例组,在过去十二个月内未发生任何急性疼痛发作的为对照组。对所有受试者评估血液学参数、HbF以及α地中海贫血的情况。
在对照组和病例组之间观察到HbF水平(P < 0.025,卡方检验)和α地中海贫血(P < 0.008,卡方检验)存在统计学显著差异。单因素分析表明,HbF水平升高> 25%(比值比:0.37,95%置信区间:0.18 - 0.77,P < 0.008)和存在α地中海贫血(比值比:0.53,95%置信区间:0.33 - 0.85,P < 0.009)具有保护作用,而多因素分析显示只有较高的HbF水平具有显著保护作用(比值比:0.39,95%置信区间:0.17 - 0.88,P < 0.025)。与对照组相比,仅在病例组的11 - 20岁年龄组中观察到显著更高的HbF水平(学生t检验,P < 0.001)。
较高浓度的HbF与预防SCD患者频繁发生急性疼痛危象有关。