Dados do Trabalho

Título

Longterm Outcome of a Kidney Transplant in Atypical Hemolytic Uremic Syndrome with Prophylatic Eculizumab: Lessons from the First Case of Terminal Complement Blockade in Latin America

Introdução

30 y old female presented with paleness, weakness and edema. Exams showed microangiopathic hemolytic anemia, thrombocytopenia and kidney injury. Serum C3 and C4 normal, autoimmune and infectious diseases were ruled out. No history of drug exposure, pregnancy or diarrhea.

Material e Método

Pulse steroids, hemodialysis and plasmapheresis/plasma infusion (PP/PI) were started leading to hematologic improvement and dialysis stop. After four months, (PP/PI) was stopped. She evolved with cholecysitis (surgery) and worsening of kidney function, resuming daily dialysis and (PP/PI) every three weeks. Haptoglobin was always undetectable, mean hemoglobin was 10.5 mg/dL and LDH was in the ULN. She also presented severe depression. Atypical Haemolytic Uremic Syndrome (aHUS) was diagnosed and genetic testing showed a variant in CFHR1, CFHR5 of alternative complement.

Resultados

She received a living donor (HLA III) kidney transplantation (tx) with prophylactic eculizumab (started 2 wk before tx). After transplantation, 4 weekly doses of eculizumab starting on day 7 ) was followed by 1200 mg biweekly from week 5. Vaccination/antibiotics were used per label. Immunosupression consisted of basiliximab, tacrolimus, mycophenolate, prednisone. Haptoglobin level normalised after the first dose of eculizumab pre-transplant. Post-transplant outcome was uneventful with no rejection or thrombotic microangiopathy. DSA three months post transplant was negative. Psychiatric condition resolved, and overall QOL improved.

Discussão e Conclusões

This case evidenced the importance of surveying subclinical activity and extrarrenal manifestations in patients with aHUS in chronic dialysis. the use of prophylactic eculizumab was associated with excellent graft outcome and no recurrence despite calcineurin inhibitor use.

Palavras Chave

Transplant, eculizumab, prophylactic, aHUS