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Cryptic activity of atypical hemolytic uremic syndrome and eculizumab treatment.

Abstract
Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease often related to uncontrolled complement activation. The use of eculizumab has changed the management and the outcome of aHUS, becoming the frontline treatment of the acute disease and for the prevention of relapses. We report the case of a male patient with aHUS due to complement factor H gene mutation who was shifted from plasmatherapy to eculizumab for preventing disease relapses. The shift to eculizumab was associated with a significant decrease in proteinuria, revealing disease activity otherwise unsuspected, being the classic criteria of disease activity (platelet, haptoglobin, LDH, schistocytes), all in the normal range.The condition of proteinuria as the only sign of thrombotic microangiopathy activity is here designated as "cryptic activity of aHUS."
AuthorsMirco Belingheri, Ilaria Possenti, Francesca Tel, Fabio Paglialonga, Sara Testa, Stefania Salardi, Gianluigi Ardissino
JournalPediatrics (Pediatrics) Vol. 133 Issue 6 Pg. e1769-71 (Jun 2014) ISSN: 1098-4275 [Electronic] United States
PMID24843058 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright © 2014 by the American Academy of Pediatrics.
Chemical References
  • Antibodies, Monoclonal, Humanized
  • Biomarkers
  • Haptoglobins
  • Complement Factor H
  • eculizumab
  • L-Lactate Dehydrogenase
Topics
  • Antibodies, Monoclonal, Humanized (adverse effects, therapeutic use)
  • Biomarkers (blood)
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Complement Activation (drug effects)
  • Complement Factor H (genetics, immunology)
  • DNA Mutational Analysis
  • Diagnosis, Differential
  • Haptoglobins (metabolism)
  • Hemolytic-Uremic Syndrome (diagnosis, drug therapy, genetics, immunology)
  • Humans
  • Infant
  • Kidney Function Tests
  • L-Lactate Dehydrogenase (blood)
  • Longitudinal Studies
  • Male
  • Plasma
  • Plasmapheresis
  • Platelet Count
  • Proteinuria (diagnosis, drug therapy, genetics, immunology)
  • Purpura, Thrombotic Thrombocytopenic (diagnosis, drug therapy, genetics, immunology)
  • Secondary Prevention

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