巨脾切除加自体脾腹膜后移植术治疗儿童重型β-地中海贫血的临床研究

Splenectomy and retroperitoneal autotransplantation of spleen in pediatric βeta-Thalassemia

目的 研究巨脾大部分切除加自体脾腹膜后移植术对儿童重型 β 地中海贫血的治疗效果及可行性分析。方法 经临床确诊的重型 β 地中海贫血并巨脾患儿 2 7例 ,其中男 1 2例 ,女 1 5例 ,年龄 4～ 1 4岁 ,平均 (6 .7± 1 .6)岁。对 1 995～ 1 999年的 1 5例患儿施行巨脾全切除术 (对照组 ) ,2 0 0 0～ 2 0 0 2年的 1 2例患儿施行脾大部分切除加自体脾腹膜后移植术 (实验组 ) ,随访半年以上者 2 4例 ,将两组的Hb平均值、血清抗体水平、99mTc 脾扫描、输血量及术后并发症等临床疗效观察进行对照分析。结果 ①术前两组Hb平均值皆约为 60 g/L左右 ,术后 1周、1个月、3个月、6个月、1年Hb先升高后呈下降趋势 ,但实验组与对照组Hb平均值差异无显著性意义 (P >0 .0 5) ;②术后 1周、3个月实验组IgM水平分别为 (0 .54± 0 .1 3)g/L和 (0 .62± 0 .1 5)g/L ,对照组为 (0 .2 2± 0 .0 5)g/L和 (0 .2 8± 0 .0 7)g/L ,实验组明显高于对照组 (P <0 .0 5) ,而IgG水平差异无显著性意义 (P >0 .0 5) ;③对照组发生OPSI(切脾后全身凶险性感染 ) 1例 ,死亡 1例 ,实验组发生切口感染、呼吸道感染等的机率明显低于对照组 ;④实验组有 6例术后 1年行99mTc 脾扫描可见脾组织显影。结论 ①脾切除术对儿童重型 β 地中海贫血有?

Objective To evaluate the effect of par ti al splenectomy and retroperitoneal autotransplantation of spleen on children wit h severe βeta-Thalassemia. Methods Twenty-seven children (12 boys and 15 girls) wit h severe βeta-Thalassemia were studied. The age ranged from 4 to 14 years ( 6.7 ± 1.6 years). Of them, 15 patients who underwent total splenectomy f rom 1995 to 1999 were served as the control group and 12 patients received part ial splenectomy with retroperitoneal autotransplantation of residual spleen betw een 2000 and 2002 were selected as experimental group. Twenty-four cases were followed-up for more than half a year. The mean value of Hb, level of IgM, Tc -99m colloid scintigraphy, volume of blood transfusion and post-operative comp lications were compared between the two groups. Results ① The mean value of Hb was about 6g % before ope ration. It increased rapidly and then decreased gradually after;There was no si gnificant difference between the two groups ( P > 0.05 ); ② The level of IgM of the experimental group was significantly higher than that of control grou p 1 week and 3 months postoperatively ( P < 0.05 ); ③ There were one case complicated with OPSI and one death in the control group. The wound infection and respiratory infection rates in the experimental group were apparently lower than that of the control; ④ Tc-99m colloid scintigraphy showed the grafted spl een could be imaged in 6 cases of the experimental group one year after transpla ntation. Conclusions Splenectomy is a safe and economic therapeuti c method for severe Thalassemia. It can decrease the side effects of hemolysis and iron overload, and reduce the regular blood transfusion. Partial splenecto my with retroperitoneal autotransplantation of spleen is an effective treatment for children with severe Thalassemia, as it not only eliminates hypersplenism, b ut also preserves immune function of spleen.