不同输血量对-地中海贫血重症患者肝铁沉着程度的影响

S. Basak, K. Das
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摘要

背景和目的:遗传性疾病(如地中海贫血)贫血患者长期输血容易导致体内铁的进行性积累。研究了β -地中海贫血重症患者肝活检切片中的铁沉积,以评估肝铁沉着的级别,并将级别与输血量联系起来。材料与方法:选取脾肿大的β -地中海贫血重症患者行脾切除术。每位患者在脾切除术时均行肝活检。肝组织切片,用珀尔斯普鲁士蓝染色观察铁沉积的存在。铁沉积程度用0 ~ 4级的铁沉着程度表示。结果:共有30名β -地中海贫血患者入组研究。30例患者中,男性7例(23.3%),女性23例(76.7%)。患者平均年龄15.2±1.4岁。研究病例的平均血清铁和铁蛋白水平高于正常范围。所有患者接受血51.5±11.6单位(范围31 ~ 88单位)。在30例患者中,1、2、3和4级肝铁沉着分别有1例、3例、9例和17例。4级患者血清铁蛋白水平显著高于3级患者(p = 0.03)。Pearson相关系数检验显示,患者肝功能分级与输血量呈正相关(0.626,p < 0.01)。结论:肝铁沉着程度与输血单位增加有关,是β -地中海贫血重症患者输血铁超载的良好指标。中华医学会医学杂志。2023。17(1): 004。DOI: https://doi.org/10.55010/imcjms.17.004*Correspondence:苏维克·巴萨克博士,普通外科,医学院,加尔各答,西孟加拉邦,印度。电子邮件:sb009cmc@gmail.com
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Grade of liver siderosis in beta-thalassaemia major patients receiving different amount of blood transfusion
Background and objectives: A progressive accumulation of body iron easily occurs as a result of long-term transfusions in patients with anaemia of genetic disorders such as thalassaemia. Iron deposit in liver biopsy sections was studied in beta-thalassaemia major patients to assess the grade of liver siderosis and to correlate the grade with amount of blood transfused. Materials and methods: Beta-thalassaemia major patients having splenomegaly and selected for splenectomy were enrolled. Liver biopsy was taken from every patient during the splenectomy. Liver tissue was sectioned and stained with Perls’ prussian blue method for the presence of iron deposition. The degree of iron deposition was expressed as grades of siderosis from 0 to 4. Results: A total of 30 beta-thalassemia patients were enrolled in the study. Out of 30 patients, 7 were males (23.3%) and 23 females (76.7%). The mean age of patients was 15.2 ± 1.4 years. The mean serum iron and ferritin levels of the study cases were above the normal range. Blood received by all patients was 51.5 ± 11.6 units (range 31 to 88 units). Out of 30 patients, grade 1, 2, 3 and 4 liver siderosis was present in 1, 3, 9 and 17 patients respectively. Serum ferritin level of patients with grade 4 siderosis was significantly higher (p = 0.03) compared to grade 3 cases. Pearson’s correlation coefficient test revealed significant positive correlation between grades of liver siderosis and amount of blood transfusion received (0.626, p < 0.01). Conclusion: Grade of liver siderosis is associated with increased units of blood transfusion and is a good indicator for transfusional iron overload in beta-thalassaemia major patients. IMC J Med Sci. 2023. 17(1): 004. DOI: https://doi.org/10.55010/imcjms.17.004 *Correspondence: Dr. Souvik Basak, Department of General Surgery, Medical College, Kolkata, West Bengal, India. Email: sb009cmc@gmail.com
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