Kida Yujiro, Ishii Toshihide, Ando Minoru, Kanda Eiichiro, Suzuki Hitoe, Kida Aiko, Yamashita Takuya, Sakamaki Hisashi, Saito Hiroshi
Department of Internal Medicine, Division of Nephrology, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
Ther Apher Dial. 2007 Oct;11(5):402-6. doi: 10.1111/j.1744-9987.2007.00504.x.
A 59-year-old-woman received related non-myeloablative allogeneic peripheral blood stem cell transplantation (PBSCT) subsequent to autologous PBSCT in our hospital five years after she was diagnosed as oligo-secretory myeloma. She was admitted to our hospital because of vomiting and grayish diarrhea 4 months after non-myeloablative allogeneic PBSCT (mini-alloPBSCT). Although her initial symptoms improved after admission, she gradually showed thrombocytopenia, anemia, and oliguria during the 2 weeks after admission. Our diagnosis was thrombotic thrombocytopenic purpura (TTP) and acute renal failure (ARF) secondary to mini-alloPBSCT. After cessation of cyclosporine administration, we began to treat her with plasma exchange (PE) and hemodialysis. During the three and a half months after we started PE, the TTP gradually improved. Although PE had been reported to be ineffective for TTP post bone marrow transplantation, we could finally discontinue PE. In contrast, since her anuria continued, she was managed with hemodialysis. One month after PE was started, her activity of von Willebrand factor-cleaving protease was 41% (normal range, >50%) and the ultrasonographic investigation of both kidneys was normal. She could be discharged after four and a half months hospitalization and lived well as an outpatient for a further two months. She died shortly after readmission from multiple organ failure without the relapse of TTP. The patient's clinical course would suggest that TTP post mini-alloPBSCT could be treated with PE in some cases, despite the development of dialysis-requiring severe ARF being a poor prognostic factor.
一名59岁女性在被诊断为少分泌型骨髓瘤五年后，于我院接受了自体外周血干细胞移植（PBSCT），之后又接受了相关的非清髓性异基因外周血干细胞移植。在非清髓性异基因PBSCT（小型异基因PBSCT）4个月后，她因呕吐和灰白色腹泻入院。入院后她的初始症状有所改善，但在入院后的2周内逐渐出现血小板减少、贫血和少尿。我们的诊断是小型异基因PBSCT继发血栓性血小板减少性紫癜（TTP）和急性肾衰竭（ARF）。停用环孢素后，我们开始用血浆置换（PE）和血液透析对她进行治疗。在开始PE后的三个半月里，TTP逐渐好转。尽管有报道称PE对骨髓移植后的TTP无效，但我们最终能够停止PE治疗。相比之下，由于她的无尿持续存在，她接受了血液透析治疗。开始PE治疗一个月后，她的血管性血友病因子裂解蛋白酶活性为41%（正常范围，>50%），双肾超声检查正常。住院四个半月后她出院了，并作为门诊病人又健康生活了两个月。再次入院后不久，她因多器官衰竭死亡，TTP未复发。该患者的临床病程表明，小型异基因PBSCT后的TTP在某些情况下可以用PE治疗，尽管需要透析的严重ARF的发生是一个不良预后因素。
