title: Long-term remission of HSCT-related NS after a second allogenic stem cell transplant.
abstract: BACKGROUND
  Hematopoietic stem cell transplantation (HSCT)-related nephrotic syndrome (NS) is a rare event and has been described as a clinical form of chronic graft-versus-host disease (GVHD). Although immunological mechanisms are thought to play important roles in NS after HSCT, the exact mechanisms have not been clarified.
  
  
  METHODS
  We report a 4-year-old boy with acute lymphoblastic leukemia (ALL) who developed NS during the tapering of immunosuppressants 5 months after an allogeneic HSCT (allo-HSCT). A renal biopsy was performed, and light and electron microscopy revealed minimal change disease (MCD). Although the response to treatment with steroids and tacrolimus was favorable, the child experienced two relapses of NS within the first 9 months after the initial response. A second allo-HSCT was performed to treat the relapse of ALL. After the second allo-HSCT, the remission of NS was maintained without recurrence for 5 years, even after the cessation of immunosuppressants.
  
  
  CONCLUSIONS
  Our patient who had ALL and developed NS after his first allo-HSCT, maintained remission from NS after a second allo-HSCT. This suggests that the immune cells from the first donor origin were associated with the pathogenesis of NS.
authors.affiliation: Department of Pediatric Oncology, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima, 960-1295, Japan. shogo@fmu.ac.jp.
authors.forename: Shogo
authors.initials: S
authors.lastname: Kobayashi
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima, 960-1295, Japan.
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Kawasaki
authors.affiliation: Department of Pediatric Oncology, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima, 960-1295, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Sano
authors.affiliation: Department of Pediatric Oncology, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima, 960-1295, Japan.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Mochizuki
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima, 960-1295, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Hosoya
authors.affiliation: Department of Pediatric Oncology, Fukushima Medical University School of Medicine, 1 Hikariga-oka, Fukushima City, Fukushima, 960-1295, Japan. akikuta@fmu.ac.jp.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kikuta
mesh_terms: D002675:Child, Preschool; D002908:Chronic Disease; D006086:Graft vs Host Disease / Q000175:diagnosis / Q000276:immunology / Q000601:surgery*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000276:immunology / Q000601:surgery*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000601:surgery*; D012008:Recurrence; D012086:Reoperation; D013997:Time Factors; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Children; Cyclosporine; FSGS; Nephrotic syndrome; Rituximab