【摘要】 目的通过总结5例活体供脾脾脏移植治疗血友病甲的经验,分析脾移植对血友病甲的治疗作用以及脾移植临床应用的方法。方法对5例血友病甲的患者分别进行亲属供脾的脾脏移植,其中父亲供脾3例,母亲供脾2例;全脾移植3例,部分脾移植2例。均采用右侧髂窝腹膜外移植,脾动脉与髂内动脉端端吻合,脾静脉与髂内或髂总静脉端侧吻合。术后采用 CsA 6～8 mg/(kg·d)+ALG 500 mg/d 或 OKT3 5～10 mg/d+Pred 或加用 Aza 1～2 mg/(kg·d)的抗排斥治疗方案。结果 5例脾移植手术均成功,术后脾脏有功能存活最短30d,最长现在已3年多。其中1例于术后38d 死于免疫抑制剂并发症,1例于术后2年因经济原因停止服用免疫抑制剂致使移植脾功能丧失,一例患者因急性排斥反应致脾破裂于术后42d 切除移植脾脏,余2例移植脾功能均良好。5例术前Ⅷ:C 浓度均<5%,术后最高达56%,最低为15%。临床出血症状完全消失,有3例术后下肢关节运动功能恢复,生活自理。结论亲属供脾脾脏移植是治疗血友病甲的有效方法,对于控制自发性出血和提高生活质量有重要的意义。更多还原

【Abstract】 Objective To summarize the experience of the treatment of hemophilia A by hving-related- donor spleen transplantation.Methods Two patients with hemophilia A underwent the living-related-donor spleen transplantation.Three donors are the patients fathers,2 are their mothers.Two patients underwent total spleen transplantation,3 underwent split spleen transplantation.Every graft was transplanted into the fight ihac fosse.The donor’s spleen artery and vein were respectively anastomosed to the recipient’s right iliac internal artery end-to-end and right iliac external vein end-to-side.The triple immunosuppressive agents of CsA 6-8 mg/(kg·d)+ALG 500 mg/d or OKT3 5- 0 mg/d+Pred or added Aza 1-2 mg/(kg·d)were administered.Results All of the opera- tions were performed successfully.The longest functional time of the graft is>3 years by now,and the shortest was 30 days.One patients was died from the side effects of immunosuppressive drugs 38 days postoperatively.One patient stopped the immunosuppressive agents because of financial problem and the non-function graft was ob- served.One graft was removed 42 days postoperatively because of acute rejection and the graft ruptured.The blood Ⅷ:C level of the 5 patients were<5% preoperatively,and rose to 15%-56% postoperatively.The hemorrhag- ic manifestation disappeared postoperatively.3 patients got good living quality.Conclusions Living-related-donor spleen transplantation is an effective method to treat hemophilia A.更多还原