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An Atypical Case of Atypical Hemolytic Uremic Syndrome

Francois, Tine, MD*; Vande Walle, Johan, PhD; Dhont, Evelyn, MD*; Keenswijk, Werner, MD†,‡

Journal of Pediatric Hematology/Oncology: March 2019 - Volume 41 - Issue 2 - p e111–e113
doi: 10.1097/MPH.0000000000001209
Online Articles: Clinical and Laboratory Observations

We present the case of a 2-month-old infant presenting with pallor and laboratory results showing: hemoglobin 5.1 (10 to 1.5) g/dL, MCV 94.7 (75 to 105) fL, leukocytes 17.4 (7 to 15) ×103/μL, platelets 259 (150 to 450) ×103/μL, hyperbilirubinemia and renal dysfunction. A hemolytic anemia with tubular injury secondary to hemoglobinuria was suspected. Hyperhydration and packed cells were given but she deteriorated. Fluid overload with anuria further complicated the course necessating hemodialysis. Atypical hemolytic uremic syndrome was suspected and eculizumab was administered resulting in rapid improvement. Genetic analysis revealed a mutation in the gene encoding complement factor H and atypical hemolytic uremic syndrome was confirmed.

Departments of *Pediatric Intensive Care

Pediatrics, Pediatric Nephrology, Ghent University Hospital, Ghent, Belgium

Department of Pediatrics, Diakonessenhuis, Paramaribo, Suriname

The authors declare no conflict of interest.

Reprints: Werner Keenswijk, MD, Department of Pediatrics, Pediatric Nephrology, Ghent University Hospital, De Pintelaan 185, Ghent 9000, Belgium (e-mail:

Received September 10, 2017

Accepted April 9, 2018

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