A TIKU1, L COYLE1, B COOPER1
1Royal North Shore Hospital, St Leonards, Australia
Background: Paroxysmal Nocturnal Haemoglobinuria (PNH) is a rare acquired blood disorder causing intravascular haemolysis and thrombophilia, and is associated with marrow hypoplasia. It can cause both acute and chronic kidney damage: respectively, through direct toxicity by free haem, and renal haemosiderosis interfering with proximal tubular function and interstitial scarring. We report a case of PNH-induced renal failure successfully managed by live kidney transplantation (4/6 HLA mismatch, no donor specific antibodies).
Case Report: Our patient was a 53-year-old man with a 20-year history of PNH. His initial treatment from diagnosis included regular blood transfusions and high dose steroids, though in more recent years requiring eculizumab every 12-14 days. Despite these treatments, he developed end stage renal failure due to progressive biopsy-proven chronic tubulo-interstitial nephritis. At time of transplant he had successfully been on home haemodialysis for five-and-a-half years. He was initially considered for combined bone marrow and kidney transplant as treatment for both PNH and ESKD, however no suitable donor was found. Peri-transplant treatments included ongoing eculizumab every 12 days, in addition to immosuppression with basiliximab and methylprednisone at induction, and tacrolimus, mycophenolate and prednisolone as maintenance. Post-transplant function was immediate, with associated fall of creatinine down to a nadir of 111 umol/L. Complications post-transplant have included a perigraft haematoma requiring surgical evacuation, an episode of steroid-responsive cellular rejection, biopsy-associated haemorrhage, drug-induced leucopaenia and neutropaenia, and occasional episodes of haemolysis associated with viral respiratory tract infections.
Conclusions: To our knowledge this is the first case of successful live renal transplantation for PNH-induced renal failure in Australia. The logistics associated with the planning for such a transplant can likely only be done with a directed live donor.
Advanced Trainee in Nephrology with the East Coast Renal Network.