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Reduced dose maintenance eculizumab in atypical hemolytic uremic syndrome (aHUS): an update on a previous case report.

ABSTRACT: To describe how maintenance eculizumab sustains improved renal function in severe atypical hemolytic uremic syndrome (aHUS).A previously described 50-year-old woman with aHUS had a remarkable recovery with eculizumab, which safely reversed profound neurologic damage and eliminated the need for dialysis. Her recovery has been sustained on long-term eculizumab treatment. She initially received eculizumab 900 mg weekly for four doses. On week 5 she commenced maintenance therapy starting at 1200 mg every 2 weeks. Due to nausea and vomiting at that dose, the maintenance dosing was reduced to 600 mg weekly, beginning on dose seven. After receiving 600 mg weekly for nine doses, eculizumab was then reduced to 600 mg every 2 weeks, with continued improvement in renal function. This dosing is lower than the usual 1200 mg every 2 weeks described in the adult literature and used in current clinical trials of aHUS.Six months after the initial diagnosis, our patient continues to have improved renal function on maintenance doses of eculizumab as low as 600 mg every 2 weeks.

SUBMITTER: Ohanian M

PROVIDER: S-EPMC3262388 | biostudies-other | 2011

REPOSITORIES: biostudies-other

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Reduced dose maintenance eculizumab in atypical hemolytic uremic syndrome (aHUS): an update on a previous case report.

Ohanian Maro M Cable Christian C Halka Kathleen K

Clinical pharmacology : advances and applications 20111101

<h4>Objective</h4>To describe how maintenance eculizumab sustains improved renal function in severe atypical hemolytic uremic syndrome (aHUS).<h4>Case report</h4>A previously described 50-year-old woman with aHUS had a remarkable recovery with eculizumab, which safely reversed profound neurologic damage and eliminated the need for dialysis. Her recovery has been sustained on long-term eculizumab treatment. She initially received eculizumab 900 mg weekly for four doses. On week 5 she commenced ma ...[more]

PMID: 22287856

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Project description:To describe a case of atypical hemolytic uremic syndrome induced by influenza A infection with the p.Ile1157Thr C3 mutation.Data sourcesClinical observations of a patient.Study selectionCase reports.Data extractionData extracted from medical records, after patient's consent.Data synthesisFour days prior to presentation to our hospital, a 16-year-old adolescent had a fever and arthralgia with hematuria. He was found to be positive for type A influenza and prescribed oseltamivir and acetaminophen by a primary-care physician. A bleeding tendency and purpura in the extremities and on the trunk developed; therefore, he was transferred to Chiba University Hospital. Hematology revealed severe thrombocytopenia, hyperbilirubinemia, and acute kidney injury. Aspartate aminotransferase, lactate dehydrogenase, and potassium could not be determined because of severe hemolysis. Highly elevated blood urea nitrogen and creatinine levels indicated acute kidney injury. A platelet count of 24,000/μL indicated thrombocytopenia, with low hemoglobin level. Peripheral blood profiling identified schistocytes. Continuous hemodiafiltration and plasma infusion were initiated immediately; however, he became oliguric. Plasma exchange was initiated on ICU day 3, but decreased urine output, hemolysis, and thrombocytopenia persisted. IV eculizumab therapy was initiated on day 7 and resulted in recovery of these symptoms and also successful discontinuation of renal support. The patient showed a stable condition without recurrence of hemolytic findings and acute kidney injury and is currently on maintenance therapy of eculizumab (1,200 mg, every other week) without any relapse of atypical hemolytic uremic syndrome symptoms. A plasma sample collected prior to initiation of plasma exchange showed an disintegrin-like and metalloprotease with thrombospondin type 1 motifs 13 activity level of 104.9%. The absence of both Shiga toxin-producing Escherichia coli in feces led to suspicion of atypical hemolytic uremic syndrome. Subsequent genetic analysis identified a mutation in C3 (p.Ile1157Thr), confirming the diagnosis of atypical hemolytic uremic syndrome.ConclusionsAlthough managing thrombocytopenia secondary to infection, inclusion of atypical hemolytic uremic syndrome in the differential diagnosis at an early stage is important in clinical practice.

Dataset Information

Reduced dose maintenance eculizumab in atypical hemolytic uremic syndrome (aHUS): an update on a previous case report.

Publications

Reduced dose maintenance eculizumab in atypical hemolytic uremic syndrome (aHUS): an update on a previous case report.

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