Lapecorella M, Napolitano M, Lucchesi A, Pisani F, Clemente K, Rizza V, Famulari A, Aloisio L, Bernardi F, Pinotti M, Ferraresi P, Di Cocco P
ASL BAT, Haematology Unit, P.O. San Nicola Pellegrino, Trani, Italy.
Transplant Proc. 2012 Sep;44(7):2033-5. doi: 10.1016/j.transproceed.2012.07.001.
Transplantation in patients with congenital bleeding disorders is a challenge requiring an integrated approach of various specialists. Renal transplantation, the most frequent type of solid organ transplantation, is rarely performed in individuals with congenital hemorrhagic disorders. We performed a renal transplantation in a 53-year-old man with end-stage renal disease and congenital coagulation factor VII deficiency, a rare bleeding disorder with a peculiar clinical picture requiring replacement therapy in surgical interventions. Perioperative bleeding was successfully prevented by administration of recombinant activated factor VII. Treatment schedule, administration rate, and long-term follow-up are reported in detail. Our report confirmed the feasibility and safety of recombinant activated factor VII in major surgical procedures like solid organ transplantations. Success requires evaluation of doses and therapeutic schedules as well as a multidisciplinary approach.
先天性出血性疾病患者的移植是一项挑战，需要多学科专家的综合治疗。肾移植是最常见的实体器官移植类型，在先天性出血性疾病患者中很少进行。我们为一名53岁的终末期肾病男性患者进行了肾移植，该患者患有先天性凝血因子VII缺乏症，这是一种罕见的出血性疾病，具有特殊的临床表现，在手术干预中需要替代治疗。通过给予重组活化因子VII成功预防了围手术期出血。详细报告了治疗方案、给药速率和长期随访情况。我们的报告证实了重组活化因子VII在实体器官移植等大型外科手术中的可行性和安全性。成功需要评估剂量和治疗方案以及多学科方法。
