title: Efficacy of Eculizumab Therapy for Atypical Hemolytic Uremic Syndrome Recurrence and Antibody-Mediated Rejection Progress After Renal Transplantation With Preformed Donor-Specific Antibodies: Case Report.
abstract: Atypical hemolytic uremic syndrome (aHUS) develops as the result of unregulated complement progression and precipitates de novo thrombotic microangiopathy. Plasma therapy is used to control the progression of the complement cascade, but that therapy is not effective in all patients and is accompanied by risk of infection and/or allergy. Eculizumab has been reported as an efficient therapy for aHUS. We report the case of a 35-year old woman who underwent effective eculizumab therapy for aHUS recurrence and antibody-mediated rejection (AMR) progress after renal transplantation with preformed donor-specific antibodies (DSA). She developed end-stage renal disease due to suspicious IgA nephropathy at age 33 years. Kidney transplantation was performed at age 35 years, and aHUS recurred 2 weeks later, leading to the progressive hemolytic anemia and renal dysfunction. Therefore, she underwent plasma therapy several times. Because it was difficult to continue to plasma therapy for severe allergy, eculizumab was proposed as an alternate therapy. Treatment with eculizumab was initiated 36 days after renal transplantation. After 3 years of eculizumab treatment, and without plasma therapy, schistocytes decreased, haptoglobin increased to within normal limits, creatinine levels stabilized, and no further episodes of diarrhea were reported. At protocol biopsy 1 year after transplantation, she was diagnosed with C4d-negative subclinical AMR. However, her pathologic findings at follow-up biopsy 3 years after transplantation were recovered. We conclude that eculizumab alone, without plasma therapy, is sufficient to treat recurrence of aHUS and AMR due to DSA after renal transplantation and to maintain long-term graft function.
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan. Electronic address: tyamamoto1976@yahoo.co.jp.
authors.forename: T
authors.initials: T
authors.lastname: Yamamoto
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Watarai
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Futamura
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Okada
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Tsujita
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Hiramitsu
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Goto
authors.affiliation: Department of Transplant Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Narumi
authors.affiliation: Department of Nephrology, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Takeda
authors.affiliation: Department of Kidney Transplant Surgery, Aichi Medical University, Nagakute, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kobayashi
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000150:complications / Q000188:drug therapy*; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D005260:Female; D005922:Glomerulonephritis, IGA / Q000150:complications; D006084:Graft Rejection / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation; D009173:Mycophenolic Acid / Q000627:therapeutic use; D011183:Postoperative Complications / Q000188:drug therapy*; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence; D016559:Tacrolimus / Q000627:therapeutic use; D014019:Tissue Donors; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D051056:Complement Inactivating Agents; D007166:Immunosuppressive Agents; D011239:Prednisolone; C481642:eculizumab; D009173:Mycophenolic Acid; D016559:Tacrolimus