Case Series of 3 Patients Diagnosed With Atypical Hemolytic Uremic Syndrome Successfully Treated With Steroids, Plasmapheresis, and Rituximab. Issue 1 (29th December 2017)

• Record Type: Journal Article
• Authors: Patterson, Jeffery M.; Bolster, Lauren; Larratt, Loree
• Is Part Of: Canadian journal of kidney health and disease =. Volume 5:Issue 1(2018)
• Publication Date: 2017-12-29
• Subjects: atypical hemolytic uremic syndrome -- thrombotic microangiopathy -- microangiopathic hemolysis -- rituximab; Kidneys -- Diseases -- Periodicals; Nephrology -- Periodicals; Dialysis -- Periodicals; Kidneys -- Transplantation -- Periodicals; Kidney Diseases -- Periodicals; Nephrology -- Periodicals; Dialysis -- Periodicals; Kidney Transplantation -- Periodicals; Dialysis; Kidneys -- Diseases; Kidneys -- Transplantation; Nephrology; Periodicals; Electronic journals; 616.61005
• DOI: 10.1177/2054358117747262
• Languages: English
• ISSNs: 2054-3581

Abstract

Rationale: Atypical hemolytic uremic syndrome, which has a high probability of chronic kidney disease, morbidity, and mortality, needs to be promptly recognized when patients present with microangiopathic hemolysis. Presenting Concerns of the Patient: Three patients present with laboratory parameters consistent with a thrombotic microangiopathy. With a suspected diagnosis of thrombotic thrombocytopenic purpura, steroids with plasmapheresis were initiated. Diagnoses: With ADAMTS13 levels reported normal, the suspected diagnoses were reevaluated. Given ongoing renal impairment, atypical hemolytic uremic syndrome was strongly considered. Interventions: When local funding issues precluded the prompt use of eculizumab, 4 doses of weekly rituximab were trialed. Outcome: Over 2 years later, all 3 patients have sustained durable remissions defined by the absence of kidney impairment or laboratory investigations concerning for microangiopathic hemolytic relapse. Lessons Learned: In cases of a suspected autoimmune mechanism leading to atypical hemolytic uremic syndrome, long-term use of eculizumab may not be required.