RESUMO
Descrever uso do Eculizumab na síndrome hemolítica urêmica atípica (SHUa) após transplante renal. Paciente de 16 anos, com diagnóstico de doença renal crônica desde 2010, decorrente de SHUa, submetida à hemodiálise. Transplante renal por doador falecido: fevereiro de 2012. Apresentou boa evolução clínica até 14º DPO, quando iniciou quadro de febre, oligúria, piora da função renal [creatinina sérica (CRs): 4,0 mg/dl] e sinais de hemólise [plaquetas: 110.000 mm3; hemoglobina (Hb): 4,5 g/dL; LDH: 3366 U/L]. Biópsia do enxerto: microangiopatia trombótica. Realizado manejo com hemoderivados (plasma fresco) e plasmaférese, com melhora da função renal (CRs: 1,46 mg/dl). Uma semana após esta intercorrência, reapresentou quadro de febre, anemia, sinais de hemólise e ITU, então manejados com ciprofloxacina, pulsoterapia com metilprednisolona e transfusão de plasma (plaquetas: 43.000 mm3; Hb: 6,0 mg/dl, reticulócitos: 1,3%, haptoglobina < 5,8 mg/dl, LDH: 1181 U/L). Após piora clínica, iniciada terapêutica com Eculizumab, 900 mg a cada cinco dias durante duas semanas. Evoluiu com boa resposta clínica, caracterizada pela melhora da função renal, normalização hematológica (plaquetas: 160.000 mm3; Hb: 11,4 g/dL) e alta hospitalar em cinco dias. Desde então, mantém uso de Eculizumab 900 mg de 15/15 dias, com quadro renal e hematológico estável. O uso de Eculizumab foi de grande utilidade no controle da recidiva da SHUa e preservação do enxerto.
To report the use of Eculizumab in atypical hemolytic uremic syndrome (aHUS) after renal transplantation. A 16 year-old patient diagnosed with chronic kidney disease since 2010, due to aHUS, under dialysis. kidney transplantation by deceased donor: February/2012. She showed good clinical evolution until the 14th postoperative day, when he developed a fever, oliguria, worsening of renal function [serum creatinine (CRs): 4.0 mg/dl] and signs of hemolysis [platelets: 110,000 mm3; hemoglobin (Hb): 4.5 g/dL; LDH: 3366 U/L]. Renal biopsy of the graft: thrombotic microangiopathy. Treated with handling blood products (fresh plasma) and plasmapheresis, with improvement of renal function (serum creatinine: 1.46 mg/dl). A week after this complication, fever anemia, signs of hemolysis and ITU restarted then it was handled with ciprofloxacin, methylprednisolone pulse therapy and plasma transfusion (Platelets: 43,000 mm3; Hb: 6.0 mg/dl, reticulocytes; 1.3%, haptoglobin < 5.8 mg/dl, HDL: 1181 U/L). After clinical worsening, it was started a therapy with Eculizumab, 900 mg in every five days for two weeks. There was some progress with good clinical response, characterized by improved renal function, stabilization of aHUS and discharged in five days. Since then, she keeps using Eculizumab 900mg each 15 days with the renal and haematological normalization (Platelets: 160,000 mm3; Hb: 11.4 g/dL). The use of Eculizumab was useful in controlling the ongoing manifestation of aHUS and transplant preservation.