title: Atypical hemolytic uremic syndrome diagnosed four years after ABO-incompatible kidney transplantation.
abstract: Atypical hemolytic uremic syndrome (aHUS) in allograft kidney transplantation is caused by various factors including rejection, infection, and immunosuppressive drugs. We present a case of a 32 year old woman with aHUS four years after an ABO-incompatible kidney transplantation from a living relative. The primary cause of end-stage renal disease was unknown; however, IgA nephropathy (IgAN) was suspected from her clinical course. She underwent pre-emptive kidney transplantation from her 60 year old mother. The allograft preserved good renal function [serum creatinine (sCr) level 110-130 μmol/L] until a sudden attack of abdominal pain four years after transplant, with acute renal failure (sCr level, 385.3 μmol/L), decreasing platelet count, and hemolytic anemia with schizocytes. On allograft biopsy, there was thrombotic microangiopathy in the glomeruli, with a cellular crescent formation and mesangial IgA and C3 deposition. Microvascular inflammation, such as glomerulitis, peritubular capillaritis, and arteriole endarteritis were also detected. A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) did not decrease and Shiga toxin was not detected. Donor-specific antibodies or autoantibodies, including anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane (anti-GBM) antibody, were negative. The patient was diagnosed with aHUS and received three sessions of plasmapheresis and methylprednisolone pulse therapy, followed by oral methylprednisolone (0.25-0.5 mg/kg) instead of tacrolimus. She temporarily required hemodialysis (sCr level, 658.3 μmol/L). Thereafter, her sCr level improved to 284.5 μmol/L without dialysis therapy. This case is clinically considered as aHUS after kidney transplantation, associated with various factors, including rejection, glomerulonephritis, and toxicity from drugs such as tacrolimus.
authors.affiliation: Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Kawaguchi
authors.affiliation: Department of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kunio
authors.initials: K
authors.lastname: Kawanishi
authors.affiliation: Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Masayo
authors.initials: M
authors.lastname: Sato
authors.affiliation: Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Mitsuyo
authors.initials: M
authors.lastname: Itabashi
authors.affiliation: Department of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Fujii
authors.affiliation: Department of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Kanetsuna
authors.affiliation: Department of Surgical Nephrology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Shouhei
authors.initials: S
authors.lastname: Huchinoue
authors.affiliation: Department of Surgical Pathology, Nippon Medical School Hospital, Tokyo, Japan.
authors.forename: Ryuji
authors.initials: R
authors.lastname: Ohashi
authors.affiliation: Department of Pathology, Kawasaki Municipal Tama Hospital, Kawasaki, Kanagawa, Japan.
authors.forename: Junki
authors.initials: J
authors.lastname: Koike
authors.affiliation: Department of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kazuho
authors.initials: K
authors.lastname: Honda
authors.affiliation: Department of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Yoji
authors.initials: Y
authors.lastname: Nagashima
authors.affiliation: Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kosaku
authors.initials: K
authors.lastname: Nitta
mesh_terms: D000017:ABO Blood-Group System / Q000276:immunology*; D000328:Adult; D065766:Atypical Hemolytic Uremic Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D001706:Biopsy; D001787:Blood Group Incompatibility / Q000276:immunology*; D046148:Donor Selection; D005260:Female; D005455:Fluorescent Antibody Technique; D006648:Histocompatibility; D006650:Histocompatibility Testing; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects* / Q000379:methods; D019520:Living Donors; D008854:Microscopy, Electron; D010956:Plasmapheresis; D006435:Renal Dialysis; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000017:ABO Blood-Group System; D007166:Immunosuppressive Agents
keywords: IgA nephropathy; atypical hemolytic uremic syndrome; kidney transplantation; necrotizing crescentic glomerulonephritis; thrombotic microangiopathy