Nakanuma Shinichi, Takamura Hiroyuki, Shoji Masatoshi, Hayashi Hironori, Tajima Hidehiro, Nakagawara Hisatoshi, Miyashita Tomoharu, Kitagawa Hirohisa, Tani Takashi, Ohta Tetsuo
From the Department of Gastroenterologic Surgery, Division of Cancer Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan.
Exp Clin Transplant. 2015 Oct;13(5):479-81. doi: 10.6002/ect.2014.0076. Epub 2014 Sep 19.
Liver transplant is a treatment for familial amyloid polyneuropathy. Few cases of ABO-incompatible living-donor liver transplant for familial amyloid polyneuropathy exist. The outcome of an ABO-incompatible living-donor liver transplant has improved recently, using local infusion therapy and rituximab prophylaxis. Here, we describe a successful ABO-incompatible living-donor liver transplant in a patient with familial amyloid polyneuropathy in whom disease progression ceased at 2 years' follow-up. Additionally, no evidence of acute or chronic rejection, or adverse events of the immunosuppressive therapy, was seen. As a postoperative complication, fatty changes in the grafted liver because of malnutrition or adverse events of corticosteroids were confirmed by a liver biopsy taken early after transplant. The main cause of malnutrition was considered to be gastrointestinal dysfunction caused by familial amyloid polyneuropathy. Therefore, before deterioration of digestive function, liver transplants should be considered for familial amyloid polyneuropathy. This case suggests that an ABO-incompatible living-donor liver transplant may provide greater opportunities for familial amyloid polyneuropathy patients.
肝移植是治疗家族性淀粉样多神经病的一种方法。家族性淀粉样多神经病的ABO血型不相容活体供肝移植病例很少。近年来,通过局部灌注治疗和利妥昔单抗预防,ABO血型不相容活体供肝移植的结果有所改善。在此,我们描述了一例家族性淀粉样多神经病患者成功进行的ABO血型不相容活体供肝移植,该患者在2年随访中疾病进展停止。此外,未发现急性或慢性排斥反应的证据,也未发现免疫抑制治疗的不良事件。作为术后并发症,移植肝脏因营养不良或皮质类固醇的不良事件出现脂肪变性,这在移植后早期进行的肝活检中得到证实。营养不良的主要原因被认为是家族性淀粉样多神经病引起的胃肠功能障碍。因此,在消化功能恶化之前,应考虑对家族性淀粉样多神经病患者进行肝移植。该病例表明,ABO血型不相容活体供肝移植可能为家族性淀粉样多神经病患者提供更多机会。