脾切除术治疗重度地中海贫血35例临床分析

Ç. Konca, Ruken Yıldırım, B. Dikici, M. Taş
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引用次数: 0

摘要

目的:地中海贫血是一种常见的、世界性的常染色体隐性和遗传性贫血,由b球蛋白合成缺失或减少引起。脾切除术可用于许多血液病的治疗。本研究的目的是讨论地中海贫血重症患者接受脾切除术的记录资料。方法:对782例重度地中海贫血患者的临床资料进行回顾性分析。在这些患者中,有35例脾切除术患者被纳入研究。对记录的患者资料进行评估,包括诊断年龄、性别、脾切除术年龄、手术指征、螯合治疗、每年输血需求和术后临床反应。采用SPSS 13.0统计软件包进行统计分析。结果:22例(63%)男性患者和13例(37%)女性患者纳入研究。平均诊断年龄为8±2个月,脾切除术年龄为8.5岁。脾切除术的主要指征是输血需求增加(57.14%)。年输血需水量由术前的235±30 mL/kg降至术后的115±15 mL/kg。脾切除术前后血清铁蛋白水平分别为1745±761 ng/mL和1985±570 ng/mL。所有患者均未出现脓毒症或凝血障碍等并发症。结论:脾切除术有利于提高患者的生活质量,显著降低患者的血耗。铁螯合治疗不充分可能与脾切除术进展有关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Splenectomy in patients with thalassemia major:Evaluation of 35 cases
Objective: Beta-thalassemia is common, worldwide, autosomal recessive and inherited type of anemia which results from absent or reduced synthesis of b globin. Splenectomy may be performed for the treatment of many hematologic diseases. The aim of this study is to discuss recorded data of thalassemia major patients who underwent splenectomy. Methods: The records of 782 patients with thalassemia major were reviewed retrospectively. Of these patients, 35 splenectomized patients with available data were included in the study. Recorded data of patients were evaluated as for age at diagnosis, gender, age at splenectomy, surgical indications, chelation therapy, annual transfusion requirements, and postoperative clinical response. Statistical analyses were performed with statistical package of SPSS version 13.0. Results: Twenty-two (63%) male and 13 (37%) female patients were included in the study. Mean age at diagnosis was 8±2 months and splenectomy was 8.5 years. The main indication for splenectomy was increased transfusion requirement (57.14%). The annual transfusion requirement reduced from preoperative 235±30 mL/kg to 115±15 mL/kg postoperatively. Mean serum ferritin levels were 1745±761 ng/mL, and 1985±570 ng/mL before, and after splenectomy. None of the patients developed any complications such as sepsis or coagulation disorder. Conclusion: These results suggest that splenectomy is beneficial to improve the quality of the lives of patients and significantly reducing blood consumption. Inadequate iron chelation therapy may be associated with progression to splenectomy.
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