Nishiwaki Kaichi, Sano Koji, Kamiyama Yutaro, Hayashi Kazumi, Tanoue Susumu, Katori Mituji, Masuoka Hidekazu, Aiba Keisuke
Division of Oncology and Hematology, Jikei University Kashiwa Hospital.
Division of Oncology and Hematology, Jikei University.
Rinsho Ketsueki. 2018;59(1):64-68. doi: 10.11406/rinketsu.59.64.
Allogeneic hematopoietic stem cell transplantation is recommended as a curative treatment option for fulminant aplastic anemia with no neutrophil despite the administration of granulocyte-colony stimulating factor. In the absence of an HLA-matched donor, unrelated cord blood transplantation (UCBT) is a treatment option that can be performed quickly. However, the optimal conditioning regimen of UCBT is yet to be established. We report two cases of fulminant aplastic anemia in adult patients who received UCBT. The first patient was a 52-year-old woman and the second was a 26-year-old man, both of whom received a conditioning regimen of total body irradiation (TBI; 2-4 Gy), fludarabine (Flu; 120 mg/kg), and cyclophosphamide (CY; 100 mg/kg) before UCBT. Short-term methotrexate and tacrolimus were used for prophylaxis of acute graft-versus-host disease (GVHD). Engraftments were achieved on days 26 and 19, and they exhibited complete donor chimerism by days 28 and 34. There was no evidence of acute GVHD, and therefore, the immunosuppressant drugs were discontinued. Reduced-intensity UCBT using a low-dose TBI/Flu/CY conditioning regimen could be an effective treatment option for fulminant aplastic anemia in the absence of a suitable donor.
对于尽管使用了粒细胞集落刺激因子但仍无中性粒细胞的暴发性再生障碍性贫血，推荐将异基因造血干细胞移植作为一种治愈性治疗选择。在没有 HLA 匹配供体的情况下，无关脐血移植（UCBT）是一种可以快速进行的治疗选择。然而，UCBT 的最佳预处理方案尚未确定。我们报告了两例接受 UCBT 的成年暴发性再生障碍性贫血患者。第一例患者是一名 52 岁女性，第二例是一名 26 岁男性，两人在 UCBT 前均接受了全身照射（TBI；2 - 4 Gy）、氟达拉滨（Flu；120 mg/kg）和环磷酰胺（CY；100 mg/kg）的预处理方案。短期使用甲氨蝶呤和他克莫司预防急性移植物抗宿主病（GVHD）。分别在第 26 天和第 19 天实现造血重建，到第 28 天和第 34 天表现出完全供体嵌合。没有急性 GVHD 的证据，因此停用了免疫抑制药物。在没有合适供体的情况下，使用低剂量 TBI/Flu/CY 预处理方案的低强度 UCBT 可能是暴发性再生障碍性贫血的一种有效治疗选择。
