尸体脾脏移植治疗血友病甲的临床观察

Clinical observation of the cadaveric spleen transplantation in the treatment of Hemophilia A

目的 探讨尸体脾移植治疗血友病甲的可行性。方法 为 1例患血友病甲的男性患儿施行了尸体脾移植手术 ,术后观察患者凝血功能、病情恢复、移植脾脏状况及免疫抑制剂浓度监测。结果 患者术后病情恢复顺利 ,无自发性出血 ,凝血功能明显改善 ,Ⅷ∶C由术前的 4%提高到术后的13%～ 2 0 % ,右下肢恢复到正常行走功能。移植脾脏未发生过排斥反应 ,亦无GVHR发生。FK5 0 6浓度调整适合 ,未见毒副作用。结论 迄今为止 ,尸体脾移植不失为治疗血友病甲的最好方法之一 ,且较亲属脾移植更为安全可行。

Objective To investigate the feasibility of cadaveric spleen transplantation in the treatment of Hemophilia A.Methods A cadaver spleen transplantation was performed on a 13 year male patient who suffered from hemophilia A.The blood coagulation function, illness recovery, the condition of the transplant spleen and FK506 blood concentration were monitored postoperatively.Results The patient recovered well after transplantation.No spontaneous bleeding occurred and blood coagulation function was obviously improved.The Ⅷ︰C was increased significantly from 4% pre operation to 13%～20% postoperation.The disabled right leg could walk freely 5 months after operation.No rejection and GVHR occurred in the period of follow up.The blood concentration of FK506 was adjusted adequately.No drug toxicity and side effects were found.Conclusion Cadaveric spleen transplantantion might be one of the best treatments for hemophilia A.This technique is safer and more feasible than living related spleen transplantation.