Introduction: KTX in aHUS patients is associated with a high rate of relapse and graft loss. Here, we report on seven aHUS cases with end-stage renal disease that were treated with peritransplant plasma exchange (PE)/infusion (PI) and ECU rescue in case of relapse/treatment intolerance.

Methods: Peritransplant PE (40ml/kg bodyweight) was carried out from days 0 to 3, then thrice weekly until stabilization of graft function followed by weekly PI (20ml/kg bodyweight). In case of intolerance/failure, ECU was started.

Results: Seven aHUS patients (mean age at first aHUS episode: 23±9 years; mean age at 1^st KTX 26±9 years) enrolled in the Vienna TMA cohort received a mean of 10±4 PE. PI was not administered in two patients (case 1, 7). Graft function remains excellent in five of our patients. Case 3 was discontinued PI after 7 years without any consequence. Four patients were switched to ECU because of (i) relapse triggered by a 3^rd pregnancy (case 4), (ii) early relapse 12 months after KTX (case 5; died 34 months later of an unknown cause), (iii) intolerance to PI (case 6) and (iv) a severe aHUS relapse triggered by antibody mediated rejection two weeks after KTX (case 7; plasma intolerance at 2^nd PE).

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Conclusion: Preemptive PE/PI is effective for prevention of aHUS relapse in patients without disease activity at the time of KTX. Five patients considered as genetically high-risk candidates showed no early recurrence of disease.

CITATION INFORMATION: Aigner C., Böhmig G., Eskandary F., Gaggl M., Kain R., Sunder-Plassmann R., Prohaszka Z., Schmidt A., Sunder-Plassmann G. Preemptive Plasma Therapy and Eculizumab (ECU) Rescue for Atypical Hemolytic Uremic Syndrome (aHUS) Relapse Following Kidney Transplantation (KTX) Am J Transplant. 2017;17 (suppl 3).

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