Thrombotic Microangiopathy in ABO-incompatible Kidney Transplant – Eculizumab to the Rescue

Abstract

A 51 year old gentleman diagnosed with end stage renal disease due to Autosomal dominant polycystic kidney disease underwent an ABO-incompatible transplant with his wife as the donor. He was given two doses of Rituximab 500mg prior to transplant and started on tacrolimus and mycophenolate mofetil fourteen days prior transplant. His baseline titres for Anti-A IgG were 1:32 and he underwent three sessions of Double filtration plasmapheresis, and pretransplant titres reduced to 1:1. Within 6 hours of the transplant surgery his urine output showed a drop and Renal angiogram showed patchy enhancement of the kidney in the interpolar region and lower pole with suspected acute kink in the arterial branch supplying the upper pole. He was re-explored in view of the kink, and an allograft biopsy was done and no kink was detected. Intraoperative renal artery doppler showed good blood flow with no evidence of thrombosis. The renal biopsy was reported to have 20-25% cortical necrosis with Vascular and Glomerular Thrombotic microangiopathy. Anti A titres were 1:16. He was subsequently treated with 7 sessions of plasmapheresis, 60 grams of IVIg and 2250mg of methylprednisolone. His urine output showed marginal improvement and was subsequently treated with two doses of 600mg Eculizumab given one week apart. Over the next few weeks his urine output improved to more than 3000ml per day and creatinine improved to 2.5mg/dl. This case highlights salvage of a hyperacute rejection with TMA with timely plasmapheresis and Eculizumab.