Partial recovery of kidney function in a case of delayed use of eculizumab in a patient with atypical hemolytic-uremic syndrome

Atypical hemolytic-uremic syndrome (aHUS) belongs to a group of thrombotic microangiopathies (TMA) characterized by profound injury to almost all organs and systems in the absence of specific treatment. Timely diagnosis and early initiation of complement-inhibiting therapy largely define the prognosis. In the presented case, aHUS was diagnosed in a 29-old female patient three weeks after the debut. The disease manifested in the context of diarrhea and resulted in progressive renal failure eventually requiring renal replacement therapy (RRT). Laboratory findings characteristic of TMA (hemolytic Coombs-negative anemia with schizocytosis, thrombocytopenia and high serum LDH) did not reach the diagnostic criteria thus the renal biopsy was required and performed. Biopsy morphology came consistent with TMA. Additional examination ruled out some secondary forms of TMA and thrombotic-thrombocytopenic purpura. At that point, aHUS diagnosis was beyond doubt. Therapeutic plasma exchange was initiated and sustained for the induction and maintenance of remission before eculizumab administration, while the patient still required dialysis. The patient also received combined antihypertensive therapy and low-molecular-weight heparin. The plasma exchange course was complicated by life-threatening allergic reactions to FFP, requiring administration of high-dose glucocorticoids. Attempts at plasma exchange cessation resulted in aHUS relapse with extrarenal symptoms - convulsions with consistent ischemic lesions in the brain structures. Plasma exchange resumption resulted in remission shortly after initiation, including resolution of ischemic brain lesions. PE dependence resulted in 8-month hospital stay, with 55 plasma exchange therapies performed. The opportunity to begin the targeted therapy presented only 13 months after the disease debut. From the time of eculizumab initiation, no relapses were registered. Renal function recovery was noted as a significant therapy achievement, allowing withdrawing the patient from dialysis, which was required for 2.5 years. RRT was terminated 15 months after eculizumab initiation. The presented case confirms the efficacy of eculizumab therapy even in the event of delayed administration.

тромботическая микроангиопатия, атипичный гемолитико-уремический синдром, почечная недостаточность, гемодиализ, плазмообмен, плазмозависимость, экулизумаб, thrombotic microangiopathy, atypical hemolytic-uremic syndrome, plasma exchange, kidney failure, hemodialysis, eculizumab

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