Ulinski T, Charpentier A, Colombat M, Desconclois C, Mougenot B, Fremaux-Bacchi V, Suberbielle C, Deschênes G, Bensman A, Veyradier A
Department of Pediatric Nephrology, Hôpital Trousseau; AP-HP & Université Paris VI, 75012 Paris, France.
Am J Transplant. 2006 Dec;6(12):3030-6. doi: 10.1111/j.1600-6143.2006.01574.x.
A 9-year-old renal transplant recipient presented with elevated serum creatinine levels 4 years post-transplant renal biopsy revealed humoral rejection including lesions suggestive for thrombotic microangiopathy (TMA). He received methylprednisolone pulses followed by a normalization of serum creatinine. Two more steroid responsive acute rejection episodes occurred. Two months later he presented rapidly progressive life threatening symptoms including bilateral pyramidal syndrome and hemoptysis. Serum haptoglobin became undetectable at this time and platelet count decreased (70000/microl), suggesting TMA. Cerebral MRI revealed generalized ischemic white matter lesions. ADAMTS13 activity decreased to < 5%. Daily plasma exchanges (PE) resulted in immediate improvement. All attempts to discontinue PE were unsuccessful. Transplantectomy resulted in normalization of generalized symptoms, hemolysis and ADAMTS13 activity (110%). Multi-organ involvement has never been reported in acquired ADAMTS13 deficiency post-transplant. Rapid resolution after transplantectomy might suggest that renal TMA was responsible for acquired ADAMTS13 deficiency and thereby triggered the generalization of TMA lesions.
一名9岁肾移植受者在移植后4年出现血清肌酐水平升高，肾活检显示体液排斥反应，包括提示血栓性微血管病（TMA）的病变。他接受了甲泼尼龙冲击治疗，随后血清肌酐恢复正常。又发生了两次对类固醇有反应的急性排斥反应。两个月后，他出现了迅速进展的危及生命的症状，包括双侧锥体束征和咯血。此时血清触珠蛋白检测不到，血小板计数下降（70000/微升），提示TMA。脑部MRI显示广泛性缺血性白质病变。ADAMTS13活性降至<5%。每日血浆置换（PE）立即改善了症状。所有停止PE的尝试均未成功。肾移植切除术使全身症状、溶血和ADAMTS13活性恢复正常（110%）。移植后获得性ADAMTS13缺乏症中多器官受累的情况此前从未有过报道。肾移植切除术后症状迅速缓解可能表明肾TMA是获得性ADAMTS13缺乏症的原因，从而引发了TMA病变的全身性表现。
