title: Favorable long-term outcome of nephrotic syndrome after allogeneic hematopoietic stem cell transplantation.
abstract: BACKGROUND
  The development of nephrotic syndrome (NS) after allogeneic hematopoietic stem cell transplantation (HS-CT) is a rare complication with few long-term outcome data.
  
  
  METHODS
  Clinical course and long-term outcome of three adult patients and one child with NS after HSCT (total number of transplants n = 533) are presented.
  
  
  RESULTS
  The median age at onset of NS was 35 years (range 15 - 56), occurring at a median of 17 months (range 11 - 21) after HSCT. Discontinuation of cyclosporine A (CSA) prior to onset of NS was a consistent feature and occurred a median of 6 months (range 2 - 10 months) prior to the development of NS. The histopathological lesion was membranous nephropathy (n = 3) and membranoproliferative glomerulonephritis Type 1 (n = 1). History of acute or concomitant clinically apparent chronic graft versus host disease (GVHD) was present in all cases except the pediatric patient who had abundant DR-activated cytotoxic T cells without evidence of viral reactivation. Long-term immunosuppression for 11 - 36 months with steroids (n = 1), combined steroids and CSA (n = 2) or CSA alone in steroid-refractory NS (n = 1) resulted in sustained remission of the NS in all patients (12 months - 8 years off immunosuppression).
  
  
  CONCLUSIONS
  NS after HSCT seems to be etiologically related to subclinical or overt chronic GVHD, which flares up after discontinuation of CSA. However, resumption of immunosuppression can reverse NS as well as GVHD and induce favorable sustained long-term remission.
authors.affiliation: Department of Pediatric Nephrology, University Children's Hospital Eppendorf, Hamburg, Germany.
authors.forename: M J
authors.initials: MJ
authors.lastname: Kemper
authors.forename: T
authors.initials: T
authors.lastname: Güngör
authors.forename: J
authors.initials: J
authors.lastname: Halter
authors.forename: U
authors.initials: U
authors.lastname: Schanz
authors.forename: T J
authors.initials: TJ
authors.lastname: Neuhaus
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000209:etiology* / Q000473:pathology; D011379:Prognosis; D014184:Transplantation, Homologous / Q000009:adverse effects
chemical_list: D007166:Immunosuppressive Agents