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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 observe the iron overload in patients with hemoglobin H disease (HbH) and to analyze the related factors. Methods 107 cases of HbH patients diagnosed by genotyping were analyzed by hematology, biochemistry and serum ferritin. Statistical analysis was performed. Results Ferritin increased in 74 (69.2%) of the 107 patients, and in 8 of 8 patients the serum ferritin was higher than 1000 ng / L. Stepwise regression analysis of age, sex, hemoglobin level, LDH levels on serum ferritin, hemoglobin levels were found to be the only independent factor (r = 0.475, P = 0.001). Serum ferritin levels were positively correlated with ALT (r = 0.596, P = 0.000), AST (r = 0.414, P = 0.000). Conclusion HbH patients, especially non-deletion type, need to be alert to iron overload. Serum ferritin and organ function should be reviewed regularly to guide the treatment.