目的观察血红蛋白H病(HbH病)患者铁超载并分析其相关因素。方法对107例经基因分型诊断HbH病的患者行血细胞分析、生化检测和血清铁蛋白检测,并行统计分析。结果 107例患者中74例(69.2%)出现铁蛋白增高,8例患者其血清铁蛋白高于1000ng/L。逐步回归法分析年龄、性别、血红蛋白水平、LDH水平对血清铁蛋白的影响,发现血红蛋白水平是唯一独立因素(r=0.475,P=0.001)。血清铁蛋白水平与ALT(r=0.596,P=0.000)、AST(r=0.414,P=0.000)水平均呈正相关。结论 HbH病患者尤其是非缺失型者需警惕铁超载,应定期复查血清铁蛋白和脏器功能以指导去铁治疗。
Objective To investigate the concentration and correlated factors of ferritin in patients with hemoglobin H(HbH) disease.Methods Red cell indices,liver function and ferritin were measured in 107 cases with HbH disease diagnosed by genotyping.Statistic analysis was carried out.Results Ferritin level increase was observed in 74 cases(69.2%),especially in 8 cases(7.4%,1 000 mg/L).Hemoglobin was suggested to be the only factor affecting ferritin with stepwise regression analysis(r = 0.475,P = 0.001).Positive correlations were revealed between ferritin and ALT(r = 0.596,P = 0.000),and AST(r = 0.414,P = 0.000).Conclusion Iron overload is common in patients with HbH disease.Ferritin level and organ function should be surveyed regularly in patients for evidences of iron chelation treatment.