title: Post-allogeneic haematopoietic stem cell transplantation membranous nephropathy: clinical presentation, outcome and pathogenic aspects.
abstract: BACKGROUND
  Post-allogeneic haematopoietic stem cell transplantation (HSCT) membranous nephropathy (MN), a rare complication of HSCT, remains an ill-defined entity. We describe the clinical and biological characteristics and outcome of five patients with post-HSCT MN, review the previously reported cases and discuss the pathogenic aspects of this nephropathy.
  
  
  METHODS
  Cases were identified by using a questionnaire sent to nephrologists and pathologists in French university and general hospitals. Medical records and kidney biopsy specimens were reviewed and relevant data were collected. Moreover, the IgG subclasses in glomerular deposits and the presence of chimeric renal cells were studied.
  
  
  RESULTS
  Five patients were identified. All had a history of chronic graft-vs-host disease (cGVHD) and all had active manifestations of cGVHD at MN diagnosis. Mean time between HSCT and diagnosis of MN was 24.4 months. Renal insufficiency was present in four patients. Renal biopsy examination showed typical features of MN in all patients. IgG1 and IgG4 were the predominant IgG subclasses in the glomerular deposits. No chimeric glomerular cell was detected. Initial treatment for MN consisted in corticosteroids and immunosuppressors (ciclosporin, mycophenolate mofetil, rituximab, chlorambucil) in all patients. Complete remission of nephrotic syndrome (NS) occurred in two patients, partial remission in one patient, while treatment was inefficacious in one (data not available for one patient). Most interestingly, the evolution of NS paralleled the evolution of cGVHD in all patients.
  
  
  CONCLUSIONS
  Our data suggest an association between cGVHD and post-HSCT MN. Treatment, mainly steroids and ciclosporin, should be aimed at the control of acute manifestations of cGVHD.
authors.affiliation: Service de Néphrologie Adultes, Hôpital Necker-Enfants Malades, AP-HP, 149-161 rue de Sèvres, 75015 Paris, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.forename: Yahsou
authors.initials: Y
authors.lastname: Delmas
authors.forename: Aurélie
authors.initials: A
authors.lastname: Hummel
authors.forename: Claire
authors.initials: C
authors.lastname: Presne
authors.forename: Francois
authors.initials: F
authors.lastname: Glowacki
authors.forename: Bertrand
authors.initials: B
authors.lastname: Knebelmann
authors.forename: Christian
authors.initials: C
authors.lastname: Combe
authors.forename: Philippe
authors.initials: P
authors.lastname: Lesavre
authors.forename: Natacha
authors.initials: N
authors.lastname: Maillard
authors.forename: Laure-Hélène
authors.initials: LH
authors.lastname: Noël
authors.forename: Natahlie
authors.initials: N
authors.lastname: Patey-Mariaud de Serre
authors.forename: Sylvie
authors.initials: S
authors.lastname: Nusbaum
authors.forename: Isabelle
authors.initials: I
authors.lastname: Radford
authors.forename: Agnès
authors.initials: A
authors.lastname: Buzyn
authors.forename: Fadi
authors.initials: F
authors.lastname: Fakhouri
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D001706:Biopsy; D002908:Chronic Disease; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D015433:Glomerulonephritis, Membranous / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology; D006086:Graft vs Host Disease / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D016572:Cyclosporine