title: Can eculizumab be discontinued in aHUS?: Case report and review of the literature.
abstract: BACKGROUND
  The management of atypical hemolytic uremic syndrome (aHUS) has evolved into better control of thrombotic microangiopathy (TMA) and recovery of renal functions since the recent introduction of the terminal complement cascade blocker, eculizumab, into clinical use. Better characterization of genotype-phenotype relations has become possible with genetic and clinical studies. However, these advances brought up some important issues, such as the possibility and timing of discontinuation of eculizumab and strategy of follow-up that need to be enlightened.
  
  
  METHODS
  One of our aHUS cases with a novel complement factor H mutation, who developed unusual laboratory findings (thrombocytopenia and mild creatinine elevation without other features of TMA) following discontinuation of eculizumab was presented. Literature and case reports relevant to discontinuation of eculizumab in aHUS patients were reviewed.
  
  
  CONCLUSIONS
  Limited experience suggests that the risk of recurrence of TMA following discontinuation of eculizumab is relatively low for patients with MCP mutations, homozygous CFHR3/R1 deletions, anti-CFH antibodies, CFI mutations, and no identifiable mutations, whereas there is a major risk for patients with CFH mutations. Early detection of TMA recurrence and prompt retreatment with eculizumab seem to be efficient in controlling of TMA and restoration of kidney functions.
authors: Department of Nephrology, Sisli Hamidiye Etfal Educational and Research Hospital, Istanbul Department of Internal Medicine, Recep Tayyip Erdogan University, Rize, Turkey Department of Nephrology, C3-P, Leiden University Medical Center, Leiden, The Netherlands.
authors: Tuncay
authors: T
authors: Sahutoglu
authors: Taner
authors: T
authors: Basturk
authors: Tamer
authors: T
authors: Sakaci
authors: Yener
authors: Y
authors: Koc
authors: Elbis
authors: E
authors: Ahbap
authors: Mustafa
authors: M
authors: Sevinc
authors: Ekrem
authors: E
authors: Kara
authors: Cuneyt
authors: C
authors: Akgol
authors: Feyza Bayraktar
authors: FB
authors: Caglayan
authors: Abdulkadir
authors: A
authors: Unsal
authors: Mohamed R
authors: MR
authors: Daha
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000009:adverse effects / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000175:diagnosis* / Q000188:drug therapy* / Q000628:therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007677:Kidney Function Tests; D057285:Precision Medicine / Q000379:methods; D006435:Renal Dialysis / Q000379:methods*; D018570:Risk Assessment; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D028761:Withholding Treatment / Q000639:trends*
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab